Materiały źródłowe

• #1 Selective IgA Deficiency – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538205/

  Immunoglobulin A (IgA) deficiency is a common primary immunodeficiency characterized by undetectable serum IgA, a concomitant lack of secretory IgA, and normal levels of other immunoglobulins. […] Like other similar immunodeficiencies, IgA deficiency appears to be a common destination to many pathogenic routes. […] Most cases of IgA deficiency appear to be sporadic. Those cases of primary IgA deficiency that appear familial in nature have no common Mendelian pattern. Secondary IgA deficiency can result from a myriad of factors. Many drugs are causative agents for IgA deficiency, including, for example, cyclosporine, gold, penicillamine, and antiepileptics. […] No matter the causative agent, the target remains the same; it is either B-cell proliferation, B-cell maturation (IgA switching), or Plasma cell production of IgA. […] Familial studies have been inconsistent; some cases appear to be autosomal dominant, while others are autosomal recessive. Chromosome mutations have been noted in JAK3, RAG1, RAG2, TAC1, CXCR4, and STAT1. […] It is thought that the overlap of genetic loci contributes to this association.

• #2 Selective IgA deficiency | Immune Deficiency Foundation

  https://primaryimmune.org/understanding-primary-immunodeficiency/types-of-pi/selective-iga-deficiency

  Selective IgA deficiency is a primary immunodeficiency characterized by an undetectable level of immunoglobulin A (IgA) in the blood and secretions but no other immunoglobulin deficiencies. […] SIgAD is defined as a primary immunodeficiency characterized by an undetectable level of IgA in the blood and secretions but normal IgG and IgM in an individual age 4 and older. […] Genetic susceptibility in IgA deficiency is not well defined, but familial inheritance of SIgAD may occur in approximately 20% of cases. […] Individuals with SIgAD lack serum (IgA 7 mg/dl) and secretory IgA, but they do make all the other immunoglobulin classes. […] Some of the individuals with SIgAD also have IgG subclass deficiency. […] In those who have associated allergic diseases, serum IgE levels may be increased.

• #3 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] It is interesting that IL-21 stimulation has been shown to induce class switch recombination to IgG and IgA and differentiation of IgA and IG secreting plasma cells with restoration of immunoglobulin production ex vivo in patients with IgA deficiency and common variable immunodeficiency (CVID).

• #4 Selective IgA deficiency | Immune Deficiency Foundation

  https://primaryimmune.org/understanding-primary-immunodeficiency/types-of-pi/selective-iga-deficiency

  Selective IgA deficiency is a primary immunodeficiency characterized by an undetectable level of immunoglobulin A (IgA) in the blood and secretions but no other immunoglobulin deficiencies. […] SIgAD is defined as a primary immunodeficiency characterized by an undetectable level of IgA in the blood and secretions but normal IgG and IgM in an individual age 4 and older. […] Genetic susceptibility in IgA deficiency is not well defined, but familial inheritance of SIgAD may occur in approximately 20% of cases. […] Individuals with SIgAD lack serum (IgA 7 mg/dl) and secretory IgA, but they do make all the other immunoglobulin classes. […] Some of the individuals with SIgAD also have IgG subclass deficiency. […] In those who have associated allergic diseases, serum IgE levels may be increased.

• #5 Selective IgA Deficiency – Immunology; Allergic Disorders – MSD Manual Professional Edition

  https://www.msdmanuals.com/professional/immunology-allergic-disorders/immunodeficiency-disorders/selective-iga-deficiency

  Selective IgA deficiency is an IgA level 7 mg/dL ( 70 mg/L, 0.4375 micromol/liter) with normal IgG and IgM levels. […] The inheritance pattern is unknown, but having a family member with selective IgA deficiency increases the risk by approximately 50 times. […] Some patients have mutations in the TACI (transmembrane activator and calcium-modulator and cyclophilin ligand interactor) gene. Selective IgA deficiency also commonly occurs in patients with certain HLA haplotypes; rare alleles or deletions of genes in the major histocompatibility complex (MHC) class III region are common. […] Medications such as phenytoin, sulfasalazine, gold, and penicillamine may lead to acquired IgA deficiency in some patients. […] Diagnosis of selective IgA deficiency is suspected in patients who have recurrent infections (including giardiasis); anaphylactic transfusion reactions; or a family history of common variable immunodeficiency (CVID), IgA deficiency, or autoimmune disorders or who are taking medications that lead to an acquired IgA deficiency.

• #6 Selective IgA Deficiency

  https://www.aaaai.org/conditions-treatments/primary-immunodeficiency-disease/selective-iga-deficiency

  Selective IgA deficiency is the most common primary immunodeficiency disease (PIDD). […] The underlying cause for Selective IgA Deficiency is unknown and there is currently no way to replace IgA in the body.

• #7 Immunoglobulin A Deficiency: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/136580-overview

  Selective immunoglobulin A deficiency (SIgAD) is a primary immunodeficiency disease and is the most common of the primary antibody deficiencies. […] IgAD is a heterogeneous disorder, and the results of intensive study are beginning to elucidate genetic loci and molecular pathogenesis that contribute to various subtypes of this disorder. […] Environmental factors such as drugs or infections can cause IgAD, but this form is reversible in more than half the cases (see Causes). […] Molecular analyses of B-cell differentiation in a small number of patients with selective or partial IgAD suggest that decreased expression level of alpha germline transcripts before a class switch might be critical for the pathogenesis of some patients with SIgAD. […] IgAD has been noted to evolve into CVID and is often observed in pedigrees that contain individuals with CVID.

• #8 Selective IgA deficiency | Immune Deficiency Foundation

  https://primaryimmune.org/understanding-primary-immunodeficiency/types-of-pi/selective-iga-deficiency

  Selective IgA deficiency is a primary immunodeficiency characterized by an undetectable level of immunoglobulin A (IgA) in the blood and secretions but no other immunoglobulin deficiencies. […] SIgAD is defined as a primary immunodeficiency characterized by an undetectable level of IgA in the blood and secretions but normal IgG and IgM in an individual age 4 and older. […] Genetic susceptibility in IgA deficiency is not well defined, but familial inheritance of SIgAD may occur in approximately 20% of cases. […] Individuals with SIgAD lack serum (IgA 7 mg/dl) and secretory IgA, but they do make all the other immunoglobulin classes. […] Some of the individuals with SIgAD also have IgG subclass deficiency. […] In those who have associated allergic diseases, serum IgE levels may be increased.

• #9 Immunoglobulin A Deficiency – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=immunoglobulin-a-deficiency-134-124

  IgA deficiency is a health problem that is passed down through families in about 1 in 5 cases. This means it is genetic. […] In rare cases, it can be caused by medicines you are taking. […] IgA deficiency is a genetic health problem that can be passed down through families.

• #10 Immunoglobulin A Deficiency Clinical Presentation: History, Physical, Causes

  https://emedicine.medscape.com/article/136580-clinical

  The underlying causes of most cases of immunoglobulin A deficiency (IgAD) remain unknown. Familial inheritance has been recognized in 20% of affected individuals, suggesting a strong genetic influence. […] Case reports of some affected families indicate that inheritance may be autosomal dominant or recessive. In other families in which multiple members are affected, the pattern of inheritance does not conform to strict Mendelian rules. […] In some families, the immunodeficiency can appear to skip generations; in others, one family member in a given generation may have IgAD, while another in the same generation may have common variable immunodeficiency (CVID), suggesting variable expressivity and penetrance of disease susceptibility gene(s). […] Studies have shown that susceptibility to either CVID or IgAD may be linked to specific alleles of the major histocompatibility complex, suggesting that these alleles, or alleles of closely linked genes with which they are in linkage disequilibrium, are somehow involved in the pathogenesis of CVID and IgAD.

• #11 Selective deficiency of IgA Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/selective-deficiency-of-iga

  Selective IgA deficiency is usually inherited, which means it is passed down through families. […] It may be inherited as an autosomal dominant or autosomal recessive trait. […] It is usually found in people of European origin. […] There are also cases of drug-induced selective IgA deficiency.

• #12 Selective deficiency of IgA: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001476.htm

  Selective IgA deficiency is usually inherited, which means it is passed down through families. […] However, there are also cases of drug-induced selective IgA deficiency. […] It may be inherited as an autosomal dominant or autosomal recessive trait. […] It is usually found in people of European origin. […] It is less common in people of other ethnicities.

• #13 Immunoglobulin A Deficiency Clinical Presentation: History, Physical, Causes

  https://emedicine.medscape.com/article/136580-clinical

  The underlying causes of most cases of immunoglobulin A deficiency (IgAD) remain unknown. Familial inheritance has been recognized in 20% of affected individuals, suggesting a strong genetic influence. […] Case reports of some affected families indicate that inheritance may be autosomal dominant or recessive. In other families in which multiple members are affected, the pattern of inheritance does not conform to strict Mendelian rules. […] In some families, the immunodeficiency can appear to skip generations; in others, one family member in a given generation may have IgAD, while another in the same generation may have common variable immunodeficiency (CVID), suggesting variable expressivity and penetrance of disease susceptibility gene(s). […] Studies have shown that susceptibility to either CVID or IgAD may be linked to specific alleles of the major histocompatibility complex, suggesting that these alleles, or alleles of closely linked genes with which they are in linkage disequilibrium, are somehow involved in the pathogenesis of CVID and IgAD.

• #14 Frontiers | Innate Mechanisms in Selective IgA Deficiency

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.649112/full

  SIgAD can occur sporadically but familial inheritance also does occur. However, no distinct Mendelian inheritance pattern is found as both autosomal recessive, autosomal dominant and sporadic inheritance were described. […] This heterogeneity in inheritance can be the result of genetic defects in different genes which are inherited in different ways, e.g. deletions in IgA1, IgA2 and IgG heavy-chain genes on chromosome 14. […] SIgAD is a disease where multiple different genetic abnormalities can be causally linked. […] The gene complex that encodes the major histocompatibility complex (MHC), in humans known as the human leukocyte antigen (HLA) complex, is located on chromosome 6. […] Certain HLA haplotypes belonging both to MHC class 1 and MHC class 2 genes, especially HLA-A1, HLA-B8, HLA-DR3 and HLA-DQ2 (collectively known as the 8.1 haplotype), is the most frequently found haplotype in individuals with IgA deficiency and could be a risk factor for SIgAD in northern parts of Europe.

• #15 Selective IgA Deficiency in Autoimmune Diseases | Molecular Medicine | Full Text

  https://molmed.biomedcentral.com/articles/10.2119/molmed.2011.00195

  Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in Caucasians. Genetic factors are important for the development of both IgAD and various autoimmune disorders, including GD, SLE, T1D, CD, MG and RA, and a strong association with the major histocompatibility complex (MHC) region has been reported. […] In addition, non-MHC genes, such as interferon-induced helicase 1 (IFIH1) and c-type lectin domain family 16, member A (CLEC16A), are also associated with the development of IgAD and some of the above diseases. This indicates a possible common genetic background. […] Interestingly, the ancestral 8.1 haplotype is also reported to be associated with Graves disease (GD), systemic lupus erythematosus (SLE), type 1 diabetes (T1D) and celiac disease (CD).

• #16 Frontiers | Innate Mechanisms in Selective IgA Deficiency

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.649112/full

  SIgAD can occur sporadically but familial inheritance also does occur. However, no distinct Mendelian inheritance pattern is found as both autosomal recessive, autosomal dominant and sporadic inheritance were described. […] This heterogeneity in inheritance can be the result of genetic defects in different genes which are inherited in different ways, e.g. deletions in IgA1, IgA2 and IgG heavy-chain genes on chromosome 14. […] SIgAD is a disease where multiple different genetic abnormalities can be causally linked. […] The gene complex that encodes the major histocompatibility complex (MHC), in humans known as the human leukocyte antigen (HLA) complex, is located on chromosome 6. […] Certain HLA haplotypes belonging both to MHC class 1 and MHC class 2 genes, especially HLA-A1, HLA-B8, HLA-DR3 and HLA-DQ2 (collectively known as the 8.1 haplotype), is the most frequently found haplotype in individuals with IgA deficiency and could be a risk factor for SIgAD in northern parts of Europe.

• #17 Selective IgA Deficiency in Autoimmune Diseases | Molecular Medicine | Full Text

  https://molmed.biomedcentral.com/articles/10.2119/molmed.2011.00195

  Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in Caucasians. Genetic factors are important for the development of both IgAD and various autoimmune disorders, including GD, SLE, T1D, CD, MG and RA, and a strong association with the major histocompatibility complex (MHC) region has been reported. […] In addition, non-MHC genes, such as interferon-induced helicase 1 (IFIH1) and c-type lectin domain family 16, member A (CLEC16A), are also associated with the development of IgAD and some of the above diseases. This indicates a possible common genetic background. […] Interestingly, the ancestral 8.1 haplotype is also reported to be associated with Graves disease (GD), systemic lupus erythematosus (SLE), type 1 diabetes (T1D) and celiac disease (CD).

• #18 Selective IgA Deficiency in Autoimmune Diseases | Molecular Medicine | Full Text

  https://molmed.biomedcentral.com/articles/10.2119/molmed.2011.00195

  It is therefore possible that IgAD and selected autoimmune disorders share some of the predisposing genes, thus explaining the increased prevalence of IgAD in certain patient groups. […] A common genetic background for selected autoimmune disorders, such as GD, SLE, T1D, CD and potentially MG and RA, involving both MHC and non-MHC encoded genes, has previously been suggested. […] The gene(s) involved are primarily located within the MHC region, where the population-attributable risk is strong in T1D, CD and RA and moderate in GD and SLE. […] Our recent preliminary work in IgAD, based on 100 multicase families, suggests a similarly strong MHC-associated risk in these patients. However, owing to strong linkage disequilibrium within the MHC region, the gene(s) involved in disease pathogenesis, with the possible exception of HLA-DQ in CD, have not yet been identified. […] It is well documented that IgAD is strongly associated with the MHC region, in particular, the HLA-B8, DR3, DQ2 haplotype. This haplotype is also associated with GD, SLE, T1D and CD.

• #19 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Mutations in transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI, TNFRSF13B) have been found both in a subset of patients with IgA deficiency or CVID. […] However, it is controversial whether TACI mutations have a cause-effect relationship with IgA deficiency or CVID. […] It is also known that IgA deficiency may progress to CVID, supporting the notion that IgA deficiency and CVID lie in the spectrum of the same disease. […] Associations between IgA deficiency and certain major histocompatibility complex (MHC) class I, II, and III haplotypes have been proposed. […] The prognosis is good in patients with IgA deficiency if it is not associated with a significant disease.

• #20 Immunoglobulin A Deficiency: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/136580-overview

  Evidence for a common pathogenesis of CVID and IgAD include shared susceptibility alleles of major histocompatibility complex class III genes (D locus). […] Previous studies of multiple-case families of patients with IgAD showed a higher prevalence of CVID among close relatives than in the general population. […] IgAD has been reported in patients with constitutional chromosome 18 abnormalities, and a case series of 83 cases of 18p- syndrome showed an increased frequency of IgAD; however, attempts to identify a specific locus on chromosome 18 have not been successful. […] The ability of many patients with SIgAD to avoid respiratory infections may relate to compensatory mechanisms at the respiratory mucosal surface and/or compensatory increases in IgG. […] The TACI receptor and 2 ligands (BAFF and APRIL) likely play a role in the pathogenesis of defective humoral immunity. […] Missense mutations in one allele of TACI were found in 4 of 19 unrelated individuals with CVID and in 1 of 16 individuals with SIgAD.

• #21 Selective IgA Deficiency in Children: Clinical Manifestations, Evaluation, and Management

  https://www.contemporarypediatrics.com/view/selective-iga-deficiency-children-clinical-manifestations-evaluation-and-management

  Selective IgA deficiency (SIGAD) is a genetic disorder with a heterogeneous clinical presentation. In children with SIGAD, B cells expressing surface IgA are present, but they are developmentally arrested. The clinical heterogeneity may be partially reflected in the extent of an arrest in B cell differentiation and, in some cases, an associated cellular immune defect. The inability of the B cells to differentiate into IgA-secreting plasma cells may be caused by the deficiency of cytokines, such as interleukin (IL)-4, IL-6, IL-7, and IL-10.7 […] A mutation in the tumor necrosis factor receptor family member TACI (transmembrane activator and calcium-modulator and cyclophilin ligand interactor) was the first genetic abnormality found in patients with SIGAD. The function of TACI is to facilitate isotype switching in B cells.8 This mutation has also been observed in some patients with CVID, which may explain why some cases of SIGAD can progress to CVID.9 Function-loss mutations in several nonmajor histocompatibility complex (MHC) genes have been found in families with SIGAD or CVID disorders, in which progression may occur from normal to CVID or to SIGAD with or without IgG subclass deficiency.10 Familial grouping of IgA deficiency has also been found; however, the pattern of inheritance has not yet been clearly identified. 11

• #22 Frontiers | Innate Mechanisms in Selective IgA Deficiency

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.649112/full

  SIgAD can occur sporadically but familial inheritance also does occur. However, no distinct Mendelian inheritance pattern is found as both autosomal recessive, autosomal dominant and sporadic inheritance were described. […] This heterogeneity in inheritance can be the result of genetic defects in different genes which are inherited in different ways, e.g. deletions in IgA1, IgA2 and IgG heavy-chain genes on chromosome 14. […] SIgAD is a disease where multiple different genetic abnormalities can be causally linked. […] The gene complex that encodes the major histocompatibility complex (MHC), in humans known as the human leukocyte antigen (HLA) complex, is located on chromosome 6. […] Certain HLA haplotypes belonging both to MHC class 1 and MHC class 2 genes, especially HLA-A1, HLA-B8, HLA-DR3 and HLA-DQ2 (collectively known as the 8.1 haplotype), is the most frequently found haplotype in individuals with IgA deficiency and could be a risk factor for SIgAD in northern parts of Europe.

• #23 Selective immunoglobulin A deficiency – Wikipedia

  https://en.wikipedia.org/wiki/Selective_immunoglobulin_A_deficiency

  Selective IgA deficiency is inherited in less than half of cases, but has been associated with differences in chromosomes 18, 14 and 6. […] Selective IgA deficiency can be inherited, but fewer than half of all cases but has been associated with some congenital intrauterine infections. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is an inherited inability to produce immunoglobulin A (IgA), a part of the body’s defenses against infection at the body’s surfaces (mainly the surfaces of the respiratory and digestive systems).

• #24 Immunoglobulin A Deficiency: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/136580-overview

  Evidence for a common pathogenesis of CVID and IgAD include shared susceptibility alleles of major histocompatibility complex class III genes (D locus). […] Previous studies of multiple-case families of patients with IgAD showed a higher prevalence of CVID among close relatives than in the general population. […] IgAD has been reported in patients with constitutional chromosome 18 abnormalities, and a case series of 83 cases of 18p- syndrome showed an increased frequency of IgAD; however, attempts to identify a specific locus on chromosome 18 have not been successful. […] The ability of many patients with SIgAD to avoid respiratory infections may relate to compensatory mechanisms at the respiratory mucosal surface and/or compensatory increases in IgG. […] The TACI receptor and 2 ligands (BAFF and APRIL) likely play a role in the pathogenesis of defective humoral immunity. […] Missense mutations in one allele of TACI were found in 4 of 19 unrelated individuals with CVID and in 1 of 16 individuals with SIgAD.

• #25 Selective IgA Deficiency – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538205/

  Immunoglobulin A (IgA) deficiency is a common primary immunodeficiency characterized by undetectable serum IgA, a concomitant lack of secretory IgA, and normal levels of other immunoglobulins. […] Like other similar immunodeficiencies, IgA deficiency appears to be a common destination to many pathogenic routes. […] Most cases of IgA deficiency appear to be sporadic. Those cases of primary IgA deficiency that appear familial in nature have no common Mendelian pattern. Secondary IgA deficiency can result from a myriad of factors. Many drugs are causative agents for IgA deficiency, including, for example, cyclosporine, gold, penicillamine, and antiepileptics. […] No matter the causative agent, the target remains the same; it is either B-cell proliferation, B-cell maturation (IgA switching), or Plasma cell production of IgA. […] Familial studies have been inconsistent; some cases appear to be autosomal dominant, while others are autosomal recessive. Chromosome mutations have been noted in JAK3, RAG1, RAG2, TAC1, CXCR4, and STAT1. […] It is thought that the overlap of genetic loci contributes to this association.

• #26 Selective IgA Deficiency | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/23356

  Like other similar immunodeficiencies, IgA deficiency appears to be a common destination to many pathogenic routes. […] Most cases of IgA deficiency appear to be sporadic. Those cases of primary IgA deficiency that appear familial in nature have no common Mendelian pattern. Secondary IgA deficiency can result from a myriad of factors. Many drugs are causative agents for IgA deficiency, including, for example, cyclosporine, gold, penicillamine, and antiepileptics. […] No matter the causative agent, the target remains the same; it is either B-cell proliferation, B-cell maturation (IgA switching), or Plasma cell production of IgA. […] Familial studies have been inconsistent; some cases appear to be autosomal dominant, while others are autosomal recessive. Chromosome mutations have been noted in JAK3, RAG1, RAG2, TAC1, CXCR4, and STAT1. […] The quality of life for the IgA deficient patient seems most affected by the disease’s indirect effects. Many patients are asymptomatic, but for others, the IgA defect remains hidden behind a facade of comorbidities.

• #27 Selective IgA Deficiency in Autoimmune Diseases | Molecular Medicine | Full Text

  https://molmed.biomedcentral.com/articles/10.2119/molmed.2011.00195

  Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in Caucasians. Genetic factors are important for the development of both IgAD and various autoimmune disorders, including GD, SLE, T1D, CD, MG and RA, and a strong association with the major histocompatibility complex (MHC) region has been reported. […] In addition, non-MHC genes, such as interferon-induced helicase 1 (IFIH1) and c-type lectin domain family 16, member A (CLEC16A), are also associated with the development of IgAD and some of the above diseases. This indicates a possible common genetic background. […] Interestingly, the ancestral 8.1 haplotype is also reported to be associated with Graves disease (GD), systemic lupus erythematosus (SLE), type 1 diabetes (T1D) and celiac disease (CD).

• #28 Selective IgA Deficiency in Autoimmune Diseases | Molecular Medicine | Full Text

  https://molmed.biomedcentral.com/articles/10.2119/molmed.2011.00195

  Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in Caucasians. Genetic factors are important for the development of both IgAD and various autoimmune disorders, including GD, SLE, T1D, CD, MG and RA, and a strong association with the major histocompatibility complex (MHC) region has been reported. […] In addition, non-MHC genes, such as interferon-induced helicase 1 (IFIH1) and c-type lectin domain family 16, member A (CLEC16A), are also associated with the development of IgAD and some of the above diseases. This indicates a possible common genetic background. […] Interestingly, the ancestral 8.1 haplotype is also reported to be associated with Graves disease (GD), systemic lupus erythematosus (SLE), type 1 diabetes (T1D) and celiac disease (CD).

• #29 Selective IgA Deficiency in Autoimmune Diseases | Molecular Medicine | Full Text

  https://molmed.biomedcentral.com/articles/10.2119/molmed.2011.00195

  It is therefore possible that IgAD and selected autoimmune disorders share some of the predisposing genes, thus explaining the increased prevalence of IgAD in certain patient groups. […] A common genetic background for selected autoimmune disorders, such as GD, SLE, T1D, CD and potentially MG and RA, involving both MHC and non-MHC encoded genes, has previously been suggested. […] The gene(s) involved are primarily located within the MHC region, where the population-attributable risk is strong in T1D, CD and RA and moderate in GD and SLE. […] Our recent preliminary work in IgAD, based on 100 multicase families, suggests a similarly strong MHC-associated risk in these patients. However, owing to strong linkage disequilibrium within the MHC region, the gene(s) involved in disease pathogenesis, with the possible exception of HLA-DQ in CD, have not yet been identified. […] It is well documented that IgAD is strongly associated with the MHC region, in particular, the HLA-B8, DR3, DQ2 haplotype. This haplotype is also associated with GD, SLE, T1D and CD.

• #30 Selective IgA Deficiency in Autoimmune Diseases | Molecular Medicine | Full Text

  https://molmed.biomedcentral.com/articles/10.2119/molmed.2011.00195

  It is therefore possible that IgAD and selected autoimmune disorders share some of the predisposing genes, thus explaining the increased prevalence of IgAD in certain patient groups. […] A common genetic background for selected autoimmune disorders, such as GD, SLE, T1D, CD and potentially MG and RA, involving both MHC and non-MHC encoded genes, has previously been suggested. […] The gene(s) involved are primarily located within the MHC region, where the population-attributable risk is strong in T1D, CD and RA and moderate in GD and SLE. […] Our recent preliminary work in IgAD, based on 100 multicase families, suggests a similarly strong MHC-associated risk in these patients. However, owing to strong linkage disequilibrium within the MHC region, the gene(s) involved in disease pathogenesis, with the possible exception of HLA-DQ in CD, have not yet been identified. […] It is well documented that IgAD is strongly associated with the MHC region, in particular, the HLA-B8, DR3, DQ2 haplotype. This haplotype is also associated with GD, SLE, T1D and CD.

• #31

  https://www.omim.org/entry/137100

  Immunoglobulin (Ig) A deficiency (IGAD) is characterized by decreased or absent levels of serum IgA in the presence of normal serum levels of IgG and IgM in a patient older than 4 years of age in whom other causes of hypogammaglobulinemia have been excluded. […] A central feature in the pathogenesis of IgA deficiency is an arrest of B-cell differentiation. Affected individuals have a normal number of IgA-bearing B-cell precursors, but a profound deficit in the terminal differentiation of IgA-secreting plasma cells. […] Hammarstrom and Smith (1999) stated that in about two-thirds of cases, selective IgA deficiency does not lead to an increased occurrence of infections, whereas the remaining patients suffer from bacterial infections in both the upper and lower respiratory tract. […] Grundbacher (1972) concluded that selective immunoglobulin A deficiency is probably multifactorial.

• #32 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] It is interesting that IL-21 stimulation has been shown to induce class switch recombination to IgG and IgA and differentiation of IgA and IG secreting plasma cells with restoration of immunoglobulin production ex vivo in patients with IgA deficiency and common variable immunodeficiency (CVID).

• #33 Selective immunoglobulin A deficiency – Wikipedia

  https://en.wikipedia.org/wiki/Selective_immunoglobulin_A_deficiency

  Selective IgA deficiency is inherited in less than half of cases, but has been associated with differences in chromosomes 18, 14 and 6. […] Selective IgA deficiency can be inherited, but fewer than half of all cases but has been associated with some congenital intrauterine infections. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is an inherited inability to produce immunoglobulin A (IgA), a part of the body’s defenses against infection at the body’s surfaces (mainly the surfaces of the respiratory and digestive systems).

• #34 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] It is interesting that IL-21 stimulation has been shown to induce class switch recombination to IgG and IgA and differentiation of IgA and IG secreting plasma cells with restoration of immunoglobulin production ex vivo in patients with IgA deficiency and common variable immunodeficiency (CVID).

• #35

  https://link.springer.com/article/10.1007/s10875-009-9357-x

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] The defect appears to involve the stem cells since IgA deficiency can be transferred by bone marrow transplantation. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] An intrinsic B cell defect, T helper cell dysfunction, and suppressor T cells have all been reported in IgA deficiency.

• #36 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] It is interesting that IL-21 stimulation has been shown to induce class switch recombination to IgG and IgA and differentiation of IgA and IG secreting plasma cells with restoration of immunoglobulin production ex vivo in patients with IgA deficiency and common variable immunodeficiency (CVID).

• #37 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  Selective IgA deficiency (SIgAD) is the most common human primary immune deficiency (PID). […] Intrinsic defects in the maturation of B cells and a perturbation of Th cells and/or cytokine signals have been hypothesized to contribute to SIgAD pathogenesis. […] The genetic basis of IgA deficiency remains to be clarified. […] Causes of isolated IgA deficiency other than SIgAD include drugs, infections, monogenic diseases and chromosomal abnormalities. […] The considerable variability in clinical manifestations suggests multiple mechanisms contributing to the pathogenesis of SIgAD. […] Intrinsic defects in the class switch recombination (CSR) mechanism and in the maturation of B cells have been reported, with decreased levels of peripheral class-switched memory B cells who cannot differentiate into IgA-secreting plasma cells.

• #38 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  Selective IgA deficiency (SIgAD) is the most common human primary immune deficiency (PID). […] Intrinsic defects in the maturation of B cells and a perturbation of Th cells and/or cytokine signals have been hypothesized to contribute to SIgAD pathogenesis. […] The genetic basis of IgA deficiency remains to be clarified. […] Causes of isolated IgA deficiency other than SIgAD include drugs, infections, monogenic diseases and chromosomal abnormalities. […] The considerable variability in clinical manifestations suggests multiple mechanisms contributing to the pathogenesis of SIgAD. […] Intrinsic defects in the class switch recombination (CSR) mechanism and in the maturation of B cells have been reported, with decreased levels of peripheral class-switched memory B cells who cannot differentiate into IgA-secreting plasma cells.

• #39 Frontiers | Innate Mechanisms in Selective IgA Deficiency

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.649112/full

  SIgAD associations were found with a major predisposing locus on chromosome 18 and structural changes including deletion of the short or the long arm or the presence of a ring-like structure of this chromosome are associated with SIgAD. […] The observation that both SIgAD and CVID can occur in families points towards a shared genetic defect based on shared susceptibility loci and alleles. […] T-cell independent IgA switching is facilitated by the presence of cytokines like BAFF (B cell activating factor) and APRIL binding to their relevant receptors TACI (transmembrane activator and calcium-modulating cyclophilin ligand interaction), BCMA (B cell maturation- antigen) and BAFF-R (BAFF-receptor). […] Defective IgA class switching might still be the main molecular defect in SIgAD.

• #40 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  Selective IgA deficiency (SIgAD) is the most common human primary immune deficiency (PID). […] Intrinsic defects in the maturation of B cells and a perturbation of Th cells and/or cytokine signals have been hypothesized to contribute to SIgAD pathogenesis. […] The genetic basis of IgA deficiency remains to be clarified. […] Causes of isolated IgA deficiency other than SIgAD include drugs, infections, monogenic diseases and chromosomal abnormalities. […] The considerable variability in clinical manifestations suggests multiple mechanisms contributing to the pathogenesis of SIgAD. […] Intrinsic defects in the class switch recombination (CSR) mechanism and in the maturation of B cells have been reported, with decreased levels of peripheral class-switched memory B cells who cannot differentiate into IgA-secreting plasma cells.

• #41 Frontiers | Innate Mechanisms in Selective IgA Deficiency

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.649112/full

  SIgAD associations were found with a major predisposing locus on chromosome 18 and structural changes including deletion of the short or the long arm or the presence of a ring-like structure of this chromosome are associated with SIgAD. […] The observation that both SIgAD and CVID can occur in families points towards a shared genetic defect based on shared susceptibility loci and alleles. […] T-cell independent IgA switching is facilitated by the presence of cytokines like BAFF (B cell activating factor) and APRIL binding to their relevant receptors TACI (transmembrane activator and calcium-modulating cyclophilin ligand interaction), BCMA (B cell maturation- antigen) and BAFF-R (BAFF-receptor). […] Defective IgA class switching might still be the main molecular defect in SIgAD.

• #42 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] It is interesting that IL-21 stimulation has been shown to induce class switch recombination to IgG and IgA and differentiation of IgA and IG secreting plasma cells with restoration of immunoglobulin production ex vivo in patients with IgA deficiency and common variable immunodeficiency (CVID).

• #43

  https://link.springer.com/article/10.1007/s10875-009-9357-x

  It is interesting that IL-21 stimulation has been shown to induce class switch recombination to IgG and IgA and differentiation of IgA and IG secreting plasma cells with restoration of immunoglobulin production ex vivo in patients with IgA deficiency and common variable immunodeficiency (CVID). […] Mutations in transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI, TNFRSF13B) have been found both in a subset of patients with IgA deficiency or CVID. […] Associations between IgA deficiency and certain major histocompatibility complex (MHC) class I, II, and III haplotypes have been proposed. […] The variation in incidence may also arise from the fact that the definition of selective IgA deficiency may differ in each study or registry.

• #44 Selective IgA deficiency // Middlesex Health

  https://middlesexhealth.org/27013558-9813-2C42-8571825DC2B165D0

  Selective IgA deficiency happens when immune system cells don’t produce any or produce very few IgA antibodies. The exact reason cells don’t produce these antibodies isn’t known. […] Certain medicines used to treat seizures, epilepsy or rheumatoid arthritis may cause selective IgA deficiency in some people. The deficiency may continue after the medicine is no longer taken. […] A family history of selective IgA deficiency increases the risk of the condition. Certain variations of genes appear to be linked to selective IgA deficiency, but no gene is known to directly cause the condition.

• #45 Selective Immunoglobulin A (IgA) Deficiency – Immune Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/immune-disorders/immunodeficiency-disorders/selective-immunoglobulin-a-iga-deficiency

  Selective immunoglobulin deficiency is usually inherited. It may be caused by a mutation in a specific gene or by a medication, such as phenytoin (used to treat seizure disorders) or sulfasalazine (used to treat rheumatoid arthritis). The inheritance pattern of the gene mutation is unknown, but having a family member with selective IgA deficiency increases the risk by about 50 times. […] It may be caused by a mutation in a specific gene or by a medication, such as phenytoin (used to treat seizure disorders) or sulfasalazine (used to treat rheumatoid arthritis). […] Some people with selective IgA deficiency produce antibodies against IgA if they are exposed to IgA in blood transfusions or immune globulin (antibodies obtained from the blood of people with a normal immune system). Rarely, these antibodies against IgA trigger a severe allergic (anaphylactic) reaction the next time such people are given a blood transfusion or immune globulin.

• #46 Selective IgA deficiency // Middlesex Health

  https://middlesexhealth.org/27013558-9813-2C42-8571825DC2B165D0

  Selective IgA deficiency happens when immune system cells don’t produce any or produce very few IgA antibodies. The exact reason cells don’t produce these antibodies isn’t known. […] Certain medicines used to treat seizures, epilepsy or rheumatoid arthritis may cause selective IgA deficiency in some people. The deficiency may continue after the medicine is no longer taken. […] A family history of selective IgA deficiency increases the risk of the condition. Certain variations of genes appear to be linked to selective IgA deficiency, but no gene is known to directly cause the condition.

• #47 Immunoglobulin A Deficiency Clinical Presentation: History, Physical, Causes

  https://emedicine.medscape.com/article/136580-clinical

  IgA deficiency is a particularly notable aspect of ataxia-telangiectasia and related disorders in which mutations in DNA processing/repair mechanisms interfere with rearrangements of immunoglobulin and T-cell receptor genes. […] In his 1991 report of two mothers with IgAD, de Laat suggests that transplacental passage of anti-IgA antibodies can also cause IgAD in an infant by inducing excessive IgA-specific T-cell suppressor activity. […] Certain drugs may also cause IgAD, but this form usually resolves once the medication is stopped. […] Infections may cause transient IgAD. The following have been recognized as causes: Rubella, Cytomegaloviruses, Toxoplasma gondii, Congenital rubella and Epstein-Barr virus infection – May result in persistent IgAD, Hepatitis C. […] IgAD can follow bone marrow transplantation from an IgA-deficient donor into a histocompatible sibling not previously deficient in IgA. […] There is also evidence that occupational exposure to benzene may contribute to IgAD.

• #48 Selective Immunoglobulin A (IgA) Deficiency – Immune Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/immune-disorders/immunodeficiency-disorders/selective-immunoglobulin-a-iga-deficiency

  Selective immunoglobulin deficiency is usually inherited. It may be caused by a mutation in a specific gene or by a medication, such as phenytoin (used to treat seizure disorders) or sulfasalazine (used to treat rheumatoid arthritis). The inheritance pattern of the gene mutation is unknown, but having a family member with selective IgA deficiency increases the risk by about 50 times. […] It may be caused by a mutation in a specific gene or by a medication, such as phenytoin (used to treat seizure disorders) or sulfasalazine (used to treat rheumatoid arthritis). […] Some people with selective IgA deficiency produce antibodies against IgA if they are exposed to IgA in blood transfusions or immune globulin (antibodies obtained from the blood of people with a normal immune system). Rarely, these antibodies against IgA trigger a severe allergic (anaphylactic) reaction the next time such people are given a blood transfusion or immune globulin.

• #49 Selective IgA Deficiency | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/23356

  Like other similar immunodeficiencies, IgA deficiency appears to be a common destination to many pathogenic routes. […] Most cases of IgA deficiency appear to be sporadic. Those cases of primary IgA deficiency that appear familial in nature have no common Mendelian pattern. Secondary IgA deficiency can result from a myriad of factors. Many drugs are causative agents for IgA deficiency, including, for example, cyclosporine, gold, penicillamine, and antiepileptics. […] No matter the causative agent, the target remains the same; it is either B-cell proliferation, B-cell maturation (IgA switching), or Plasma cell production of IgA. […] Familial studies have been inconsistent; some cases appear to be autosomal dominant, while others are autosomal recessive. Chromosome mutations have been noted in JAK3, RAG1, RAG2, TAC1, CXCR4, and STAT1. […] The quality of life for the IgA deficient patient seems most affected by the disease’s indirect effects. Many patients are asymptomatic, but for others, the IgA defect remains hidden behind a facade of comorbidities.

• #50 Selective IgA Deficiency in Children: Clinical Manifestations, Evaluation, and Management

  https://www.contemporarypediatrics.com/view/selective-iga-deficiency-children-clinical-manifestations-evaluation-and-management

  SIGAD also may be associated with deficiencies in antipneumococcal antibodies. Drugs. A large number of medications may cause SIGAD, which usually resolves when the medication is discontinued. Examples of these medications include phenytoin, carbamazepine, valproic acid, zonisamide, sulfasalazine, gold compounds, D-penicillamine, hydroxychloroquine, NSAIDs, captopril, and thyroxine.1 In addition, treatment of uveitis with cyclosporin A has been reported to cause permanent IgA deficiency.12

• #51 Selective IgA Deficiency – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538205/

  Immunoglobulin A (IgA) deficiency is a common primary immunodeficiency characterized by undetectable serum IgA, a concomitant lack of secretory IgA, and normal levels of other immunoglobulins. […] Like other similar immunodeficiencies, IgA deficiency appears to be a common destination to many pathogenic routes. […] Most cases of IgA deficiency appear to be sporadic. Those cases of primary IgA deficiency that appear familial in nature have no common Mendelian pattern. Secondary IgA deficiency can result from a myriad of factors. Many drugs are causative agents for IgA deficiency, including, for example, cyclosporine, gold, penicillamine, and antiepileptics. […] No matter the causative agent, the target remains the same; it is either B-cell proliferation, B-cell maturation (IgA switching), or Plasma cell production of IgA. […] Familial studies have been inconsistent; some cases appear to be autosomal dominant, while others are autosomal recessive. Chromosome mutations have been noted in JAK3, RAG1, RAG2, TAC1, CXCR4, and STAT1. […] It is thought that the overlap of genetic loci contributes to this association.

• #52 Selective IgA Deficiency | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/23356

  Like other similar immunodeficiencies, IgA deficiency appears to be a common destination to many pathogenic routes. […] Most cases of IgA deficiency appear to be sporadic. Those cases of primary IgA deficiency that appear familial in nature have no common Mendelian pattern. Secondary IgA deficiency can result from a myriad of factors. Many drugs are causative agents for IgA deficiency, including, for example, cyclosporine, gold, penicillamine, and antiepileptics. […] No matter the causative agent, the target remains the same; it is either B-cell proliferation, B-cell maturation (IgA switching), or Plasma cell production of IgA. […] Familial studies have been inconsistent; some cases appear to be autosomal dominant, while others are autosomal recessive. Chromosome mutations have been noted in JAK3, RAG1, RAG2, TAC1, CXCR4, and STAT1. […] The quality of life for the IgA deficient patient seems most affected by the disease’s indirect effects. Many patients are asymptomatic, but for others, the IgA defect remains hidden behind a facade of comorbidities.

• #53 Immunoglobulin A Deficiency Clinical Presentation: History, Physical, Causes

  https://emedicine.medscape.com/article/136580-clinical

  IgA deficiency is a particularly notable aspect of ataxia-telangiectasia and related disorders in which mutations in DNA processing/repair mechanisms interfere with rearrangements of immunoglobulin and T-cell receptor genes. […] In his 1991 report of two mothers with IgAD, de Laat suggests that transplacental passage of anti-IgA antibodies can also cause IgAD in an infant by inducing excessive IgA-specific T-cell suppressor activity. […] Certain drugs may also cause IgAD, but this form usually resolves once the medication is stopped. […] Infections may cause transient IgAD. The following have been recognized as causes: Rubella, Cytomegaloviruses, Toxoplasma gondii, Congenital rubella and Epstein-Barr virus infection – May result in persistent IgAD, Hepatitis C. […] IgAD can follow bone marrow transplantation from an IgA-deficient donor into a histocompatible sibling not previously deficient in IgA. […] There is also evidence that occupational exposure to benzene may contribute to IgAD.

• #54 Selective IgA deficiency – GPnotebook

  https://gpnotebook.com/pages/infectious-disease/selective-iga-deficiency

  Virtual absence of serum and secretory IgA, and occasionally of IgG2 and IgG4 is relatively common, affecting 1 in 600 people. It may be familial or may be acquired following toxoplasmosis, measles, or other viral infections. […] The basic defect is a failure of maturation of IgA positive B cells.

• #55 Low IgA and Selective IgA Deficiency

  https://www.geneticlifehacks.com/low-iga-and-selective-deficiency/

  Selective IgA deficiency can be (partly) genetic, or it can be due to other causes such as medications or autoimmunity. […] IgA deficiency is a genetic immunodeficiency disorder characterized by the absence or very low levels of immunoglobulin A (IgA). […] One reason for low IgA is depletion of CD4+CD25+ Treg cells. Another is the inability of B cells to switch classes. Yet another mechanism is reduced proliferative activity of CD4+ T cells. […] IgA deficiency can be inherited, and there is a genetic component to it for most people. […] Certain HLA types are associated with an increased susceptibility to IgA deficiency, and mutations related to common variable immunodeficiency can also cause IgA deficiency. […] Certain bacteria and viruses can secrete an enzyme that destroys IgA, resulting in an IgA deficiency during infection. […] IgA deficiency may also be autoimmune or caused by certain medications.

• #56 Immunoglobulin A Deficiency Clinical Presentation: History, Physical, Causes

  https://emedicine.medscape.com/article/136580-clinical

  IgA deficiency is a particularly notable aspect of ataxia-telangiectasia and related disorders in which mutations in DNA processing/repair mechanisms interfere with rearrangements of immunoglobulin and T-cell receptor genes. […] In his 1991 report of two mothers with IgAD, de Laat suggests that transplacental passage of anti-IgA antibodies can also cause IgAD in an infant by inducing excessive IgA-specific T-cell suppressor activity. […] Certain drugs may also cause IgAD, but this form usually resolves once the medication is stopped. […] Infections may cause transient IgAD. The following have been recognized as causes: Rubella, Cytomegaloviruses, Toxoplasma gondii, Congenital rubella and Epstein-Barr virus infection – May result in persistent IgAD, Hepatitis C. […] IgAD can follow bone marrow transplantation from an IgA-deficient donor into a histocompatible sibling not previously deficient in IgA. […] There is also evidence that occupational exposure to benzene may contribute to IgAD.

• #57 IgA Deficiency – AmeriPharma® Specialty Care

  https://ameripharmaspecialty.com/other-health-conditions/iga-deficiency/

  Apart from IgA, there are other antibodies responsible for protecting us against infections, such as the IgG and IgM antibodies. IgA deficiency (also known as selective IgA deficiency) is when a person has low or nonexistent levels of IgA in their blood. It is considered a primary immunodeficiency. […] Several different factors can contribute to low IgA levels in the blood, including: […] Certain health conditions are responsible for causing low IgA levels. For example, poor sleep, gut disorders, exhaustion, allergies, and chronic stress can lower IgA levels in saliva. […] Autoimmune diseases (when your immune system attacks its own tissues and organs) may also decrease IgA levels. Autoimmune diseases that may decrease IgA levels include: […] Hereditary factors also play a role in causing low IgA levels. Although rare, those with a family history of IgA deficiency are more likely to develop low IgA levels.

• #58 Iga Deficiency: Causes, Symptoms And Treatment

  https://www.medicoverhospitals.in/diseases/iga-deficiency/

  Genetic factors such as inherited mutations in the genes responsible for producing IgA antibodies can lead to IgA deficiency. […] Additionally, environmental factors and family history may also contribute to the development of IgA deficiency. […] Certain autoimmune conditions, such as rheumatoid arthritis or systemic lupus erythematosus, may be associated with IgA deficiency. […] Some medications, including certain antibiotics or anticonvulsants, have been linked to the development of IgA deficiency in some individuals. […] Chronic infections, particularly of the respiratory or gastrointestinal tracts, can trigger an immune response that disrupts the production of IgA antibodies. […] Conditions that affect the gastrointestinal system, such as celiac disease or inflammatory bowel disease, are known to be correlated with IgA deficiency.

• #59 Immunoglobulin A Deficiency Clinical Presentation: History, Physical, Causes

  https://emedicine.medscape.com/article/136580-clinical

  IgA deficiency is a particularly notable aspect of ataxia-telangiectasia and related disorders in which mutations in DNA processing/repair mechanisms interfere with rearrangements of immunoglobulin and T-cell receptor genes. […] In his 1991 report of two mothers with IgAD, de Laat suggests that transplacental passage of anti-IgA antibodies can also cause IgAD in an infant by inducing excessive IgA-specific T-cell suppressor activity. […] Certain drugs may also cause IgAD, but this form usually resolves once the medication is stopped. […] Infections may cause transient IgAD. The following have been recognized as causes: Rubella, Cytomegaloviruses, Toxoplasma gondii, Congenital rubella and Epstein-Barr virus infection – May result in persistent IgAD, Hepatitis C. […] IgAD can follow bone marrow transplantation from an IgA-deficient donor into a histocompatible sibling not previously deficient in IgA. […] There is also evidence that occupational exposure to benzene may contribute to IgAD.

• #60

  https://link.springer.com/article/10.1007/s10875-009-9357-x

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] The defect appears to involve the stem cells since IgA deficiency can be transferred by bone marrow transplantation. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] An intrinsic B cell defect, T helper cell dysfunction, and suppressor T cells have all been reported in IgA deficiency.

• #61 Immunoglobulin A Deficiency Clinical Presentation: History, Physical, Causes

  https://emedicine.medscape.com/article/136580-clinical

  IgA deficiency is a particularly notable aspect of ataxia-telangiectasia and related disorders in which mutations in DNA processing/repair mechanisms interfere with rearrangements of immunoglobulin and T-cell receptor genes. […] In his 1991 report of two mothers with IgAD, de Laat suggests that transplacental passage of anti-IgA antibodies can also cause IgAD in an infant by inducing excessive IgA-specific T-cell suppressor activity. […] Certain drugs may also cause IgAD, but this form usually resolves once the medication is stopped. […] Infections may cause transient IgAD. The following have been recognized as causes: Rubella, Cytomegaloviruses, Toxoplasma gondii, Congenital rubella and Epstein-Barr virus infection – May result in persistent IgAD, Hepatitis C. […] IgAD can follow bone marrow transplantation from an IgA-deficient donor into a histocompatible sibling not previously deficient in IgA. […] There is also evidence that occupational exposure to benzene may contribute to IgAD.

• #62 IgA Deficiency – AmeriPharma® Specialty Care

  https://ameripharmaspecialty.com/other-health-conditions/iga-deficiency/

  Apart from IgA, there are other antibodies responsible for protecting us against infections, such as the IgG and IgM antibodies. IgA deficiency (also known as selective IgA deficiency) is when a person has low or nonexistent levels of IgA in their blood. It is considered a primary immunodeficiency. […] Several different factors can contribute to low IgA levels in the blood, including: […] Certain health conditions are responsible for causing low IgA levels. For example, poor sleep, gut disorders, exhaustion, allergies, and chronic stress can lower IgA levels in saliva. […] Autoimmune diseases (when your immune system attacks its own tissues and organs) may also decrease IgA levels. Autoimmune diseases that may decrease IgA levels include: […] Hereditary factors also play a role in causing low IgA levels. Although rare, those with a family history of IgA deficiency are more likely to develop low IgA levels.

• #63 Iga Deficiency: Causes, Symptoms And Treatment

  https://www.medicoverhospitals.in/diseases/iga-deficiency/

  Genetic factors such as inherited mutations in the genes responsible for producing IgA antibodies can lead to IgA deficiency. […] Additionally, environmental factors and family history may also contribute to the development of IgA deficiency. […] Certain autoimmune conditions, such as rheumatoid arthritis or systemic lupus erythematosus, may be associated with IgA deficiency. […] Some medications, including certain antibiotics or anticonvulsants, have been linked to the development of IgA deficiency in some individuals. […] Chronic infections, particularly of the respiratory or gastrointestinal tracts, can trigger an immune response that disrupts the production of IgA antibodies. […] Conditions that affect the gastrointestinal system, such as celiac disease or inflammatory bowel disease, are known to be correlated with IgA deficiency.

• #64 Iga Deficiency: Causes, Symptoms And Treatment

  https://www.medicoverhospitals.in/diseases/iga-deficiency/

  Genetic factors such as inherited mutations in the genes responsible for producing IgA antibodies can lead to IgA deficiency. […] Additionally, environmental factors and family history may also contribute to the development of IgA deficiency. […] Certain autoimmune conditions, such as rheumatoid arthritis or systemic lupus erythematosus, may be associated with IgA deficiency. […] Some medications, including certain antibiotics or anticonvulsants, have been linked to the development of IgA deficiency in some individuals. […] Chronic infections, particularly of the respiratory or gastrointestinal tracts, can trigger an immune response that disrupts the production of IgA antibodies. […] Conditions that affect the gastrointestinal system, such as celiac disease or inflammatory bowel disease, are known to be correlated with IgA deficiency.

• #65 IgA Deficiency – AmeriPharma® Specialty Care

  https://ameripharmaspecialty.com/other-health-conditions/iga-deficiency/

  Apart from IgA, there are other antibodies responsible for protecting us against infections, such as the IgG and IgM antibodies. IgA deficiency (also known as selective IgA deficiency) is when a person has low or nonexistent levels of IgA in their blood. It is considered a primary immunodeficiency. […] Several different factors can contribute to low IgA levels in the blood, including: […] Certain health conditions are responsible for causing low IgA levels. For example, poor sleep, gut disorders, exhaustion, allergies, and chronic stress can lower IgA levels in saliva. […] Autoimmune diseases (when your immune system attacks its own tissues and organs) may also decrease IgA levels. Autoimmune diseases that may decrease IgA levels include: […] Hereditary factors also play a role in causing low IgA levels. Although rare, those with a family history of IgA deficiency are more likely to develop low IgA levels.

• #66 Immunoglobulin A Deficiency Clinical Presentation: History, Physical, Causes

  https://emedicine.medscape.com/article/136580-clinical

  IgA deficiency is a particularly notable aspect of ataxia-telangiectasia and related disorders in which mutations in DNA processing/repair mechanisms interfere with rearrangements of immunoglobulin and T-cell receptor genes. […] In his 1991 report of two mothers with IgAD, de Laat suggests that transplacental passage of anti-IgA antibodies can also cause IgAD in an infant by inducing excessive IgA-specific T-cell suppressor activity. […] Certain drugs may also cause IgAD, but this form usually resolves once the medication is stopped. […] Infections may cause transient IgAD. The following have been recognized as causes: Rubella, Cytomegaloviruses, Toxoplasma gondii, Congenital rubella and Epstein-Barr virus infection – May result in persistent IgAD, Hepatitis C. […] IgAD can follow bone marrow transplantation from an IgA-deficient donor into a histocompatible sibling not previously deficient in IgA. […] There is also evidence that occupational exposure to benzene may contribute to IgAD.

• #67 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Mutations in transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI, TNFRSF13B) have been found both in a subset of patients with IgA deficiency or CVID. […] However, it is controversial whether TACI mutations have a cause-effect relationship with IgA deficiency or CVID. […] It is also known that IgA deficiency may progress to CVID, supporting the notion that IgA deficiency and CVID lie in the spectrum of the same disease. […] Associations between IgA deficiency and certain major histocompatibility complex (MHC) class I, II, and III haplotypes have been proposed. […] The prognosis is good in patients with IgA deficiency if it is not associated with a significant disease.

• #68 Immunoglobulin A Deficiency: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/136580-overview

  Selective immunoglobulin A deficiency (SIgAD) is a primary immunodeficiency disease and is the most common of the primary antibody deficiencies. […] IgAD is a heterogeneous disorder, and the results of intensive study are beginning to elucidate genetic loci and molecular pathogenesis that contribute to various subtypes of this disorder. […] Environmental factors such as drugs or infections can cause IgAD, but this form is reversible in more than half the cases (see Causes). […] Molecular analyses of B-cell differentiation in a small number of patients with selective or partial IgAD suggest that decreased expression level of alpha germline transcripts before a class switch might be critical for the pathogenesis of some patients with SIgAD. […] IgAD has been noted to evolve into CVID and is often observed in pedigrees that contain individuals with CVID.

• #69 Immunoglobulin A Deficiency Clinical Presentation: History, Physical, Causes

  https://emedicine.medscape.com/article/136580-clinical

  The underlying causes of most cases of immunoglobulin A deficiency (IgAD) remain unknown. Familial inheritance has been recognized in 20% of affected individuals, suggesting a strong genetic influence. […] Case reports of some affected families indicate that inheritance may be autosomal dominant or recessive. In other families in which multiple members are affected, the pattern of inheritance does not conform to strict Mendelian rules. […] In some families, the immunodeficiency can appear to skip generations; in others, one family member in a given generation may have IgAD, while another in the same generation may have common variable immunodeficiency (CVID), suggesting variable expressivity and penetrance of disease susceptibility gene(s). […] Studies have shown that susceptibility to either CVID or IgAD may be linked to specific alleles of the major histocompatibility complex, suggesting that these alleles, or alleles of closely linked genes with which they are in linkage disequilibrium, are somehow involved in the pathogenesis of CVID and IgAD.

• #70 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Mutations in transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI, TNFRSF13B) have been found both in a subset of patients with IgA deficiency or CVID. […] However, it is controversial whether TACI mutations have a cause-effect relationship with IgA deficiency or CVID. […] It is also known that IgA deficiency may progress to CVID, supporting the notion that IgA deficiency and CVID lie in the spectrum of the same disease. […] Associations between IgA deficiency and certain major histocompatibility complex (MHC) class I, II, and III haplotypes have been proposed. […] The prognosis is good in patients with IgA deficiency if it is not associated with a significant disease.

• #71 Selective IgA Deficiency – Immunology; Allergic Disorders – MSD Manual Professional Edition

  https://www.msdmanuals.com/professional/immunology-allergic-disorders/immunodeficiency-disorders/selective-iga-deficiency

  Because immune globulin replacement therapy contains mostly IgG and minimal amounts of IgA, patients with IgA deficiency do not benefit from it. However, there still is some risk of sensitizing patients to IgA or triggering an anaphylactic reaction in those who previously developed anti-IgA antibodies. […] A few patients with IgA deficiency develop CVID over time; others improve spontaneously.

• #72 Immunoglobulin A Deficiency: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/136580-overview

  Evidence for a common pathogenesis of CVID and IgAD include shared susceptibility alleles of major histocompatibility complex class III genes (D locus). […] Previous studies of multiple-case families of patients with IgAD showed a higher prevalence of CVID among close relatives than in the general population. […] IgAD has been reported in patients with constitutional chromosome 18 abnormalities, and a case series of 83 cases of 18p- syndrome showed an increased frequency of IgAD; however, attempts to identify a specific locus on chromosome 18 have not been successful. […] The ability of many patients with SIgAD to avoid respiratory infections may relate to compensatory mechanisms at the respiratory mucosal surface and/or compensatory increases in IgG. […] The TACI receptor and 2 ligands (BAFF and APRIL) likely play a role in the pathogenesis of defective humoral immunity. […] Missense mutations in one allele of TACI were found in 4 of 19 unrelated individuals with CVID and in 1 of 16 individuals with SIgAD.

• #73 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Mutations in transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI, TNFRSF13B) have been found both in a subset of patients with IgA deficiency or CVID. […] However, it is controversial whether TACI mutations have a cause-effect relationship with IgA deficiency or CVID. […] It is also known that IgA deficiency may progress to CVID, supporting the notion that IgA deficiency and CVID lie in the spectrum of the same disease. […] Associations between IgA deficiency and certain major histocompatibility complex (MHC) class I, II, and III haplotypes have been proposed. […] The prognosis is good in patients with IgA deficiency if it is not associated with a significant disease.

• #74 Frontiers | Innate Mechanisms in Selective IgA Deficiency

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.649112/full

  SIgAD associations were found with a major predisposing locus on chromosome 18 and structural changes including deletion of the short or the long arm or the presence of a ring-like structure of this chromosome are associated with SIgAD. […] The observation that both SIgAD and CVID can occur in families points towards a shared genetic defect based on shared susceptibility loci and alleles. […] T-cell independent IgA switching is facilitated by the presence of cytokines like BAFF (B cell activating factor) and APRIL binding to their relevant receptors TACI (transmembrane activator and calcium-modulating cyclophilin ligand interaction), BCMA (B cell maturation- antigen) and BAFF-R (BAFF-receptor). […] Defective IgA class switching might still be the main molecular defect in SIgAD.

• #75 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Mutations in transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI, TNFRSF13B) have been found both in a subset of patients with IgA deficiency or CVID. […] However, it is controversial whether TACI mutations have a cause-effect relationship with IgA deficiency or CVID. […] It is also known that IgA deficiency may progress to CVID, supporting the notion that IgA deficiency and CVID lie in the spectrum of the same disease. […] Associations between IgA deficiency and certain major histocompatibility complex (MHC) class I, II, and III haplotypes have been proposed. […] The prognosis is good in patients with IgA deficiency if it is not associated with a significant disease.

• #76 Incident — Case 280

  https://path.upmc.edu/cases/case280/inc.html

  IgAD is exemplified by serum IgA level of Common variable immunodeficiency (CVID), the most common form of primary panhypogammaglobulinemia, affects 1 in 50,000 Caucasians and is characterized by a marked reduction in serum levels of IgG ( Both IgAD and CVID can follow exposure to anti-rheumatic and anti-epileptic medications. Other secondary causes include viral infections, notably rubella. Some cases of CVID develop from IgAD and occasionally IgAD has been known to develop from CVID. Moreover, anti-IgA antibodies can be detected in both diseases. Some investigators believe these disorders to represent the polar ends of a clinical spectrum of humoral immunodeficiency, reflecting a single underlying genetic defect. […] While the genetic factors underlying these disorders are not exactly known, the defect appears to involve immunoglobulin class switching at the stem cell level.

• #77 Selective deficiency of IgA: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001476.htm

  Selective IgA deficiency is usually inherited, which means it is passed down through families. […] However, there are also cases of drug-induced selective IgA deficiency. […] It may be inherited as an autosomal dominant or autosomal recessive trait. […] It is usually found in people of European origin. […] It is less common in people of other ethnicities.

• #78 Selective deficiency of IgA Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/selective-deficiency-of-iga

  Selective IgA deficiency is usually inherited, which means it is passed down through families. […] It may be inherited as an autosomal dominant or autosomal recessive trait. […] It is usually found in people of European origin. […] There are also cases of drug-induced selective IgA deficiency.

• #79 Selective Deficiency of IgA – UF Health

  https://ufhealth.org/conditions-and-treatments/selective-deficiency-of-iga

  Selective deficiency of IgA is usually inherited, which means it is passed down through families. […] It may be inherited as an autosomal dominant or autosomal recessive trait. […] It is usually found in people of European origin. It is less common in people of other ethnicities.

• #80 Selective deficiency of IgA: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001476.htm

  Selective IgA deficiency is usually inherited, which means it is passed down through families. […] However, there are also cases of drug-induced selective IgA deficiency. […] It may be inherited as an autosomal dominant or autosomal recessive trait. […] It is usually found in people of European origin. […] It is less common in people of other ethnicities.

• #81 Low IgA & Selective IgA Deficiency: Causes & Symptoms – SelfDecode Labs

  https://labs.selfdecode.com/blog/iga-deficiency/

  Low IgA can be caused by selective IgA deficiency, when only IgA levels are low but other antibodies (IgG, IgM, IgD, and IgE) are normal. […] Selective IgA deficiency can be inherited or occur spontaneously (due to infections, medication, or unknown causes). The genetic disorder that causes people to lack IgA is found in up to 1% of the population. […] People are IgA deficient if they have blood IgA levels below 7 mg/dL with normal IgM and IgG levels. […] Selective IgA deficiency is when IgA levels are 7 mg/dL but IgM and IgG are normal. It can be caused by genetic disorders and non-genetic factors. […] Patients with selective IgA deficiency (undetectable IgA but normal IgG and IgM levels) have a 10 to 20 times higher risk of celiac disease. […] People with IgA deficiency have a higher association of type 1 diabetes (up to 10 times), lupus, juvenile idiopathic arthritis, IBD (both Crohns and ulcerative colitis), rheumatoid arthritis, and thyroid disorders. […] Some studies have associated IgA deficiency with IBD and IBS, though the research is still inconclusive. […] Those with IgA deficiency have a moderately increased risk of cancer, especially gut cancer.

• #82 Selective deficiency of IgA: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001476.htm

  Selective IgA deficiency is usually inherited, which means it is passed down through families. […] However, there are also cases of drug-induced selective IgA deficiency. […] It may be inherited as an autosomal dominant or autosomal recessive trait. […] It is usually found in people of European origin. […] It is less common in people of other ethnicities.

• #83 Frontiers | Innate Mechanisms in Selective IgA Deficiency

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.649112/full

  SIgAD can occur sporadically but familial inheritance also does occur. However, no distinct Mendelian inheritance pattern is found as both autosomal recessive, autosomal dominant and sporadic inheritance were described. […] This heterogeneity in inheritance can be the result of genetic defects in different genes which are inherited in different ways, e.g. deletions in IgA1, IgA2 and IgG heavy-chain genes on chromosome 14. […] SIgAD is a disease where multiple different genetic abnormalities can be causally linked. […] The gene complex that encodes the major histocompatibility complex (MHC), in humans known as the human leukocyte antigen (HLA) complex, is located on chromosome 6. […] Certain HLA haplotypes belonging both to MHC class 1 and MHC class 2 genes, especially HLA-A1, HLA-B8, HLA-DR3 and HLA-DQ2 (collectively known as the 8.1 haplotype), is the most frequently found haplotype in individuals with IgA deficiency and could be a risk factor for SIgAD in northern parts of Europe.

• #84

  https://link.springer.com/article/10.1007/s10875-009-9357-x

  It is interesting that IL-21 stimulation has been shown to induce class switch recombination to IgG and IgA and differentiation of IgA and IG secreting plasma cells with restoration of immunoglobulin production ex vivo in patients with IgA deficiency and common variable immunodeficiency (CVID). […] Mutations in transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI, TNFRSF13B) have been found both in a subset of patients with IgA deficiency or CVID. […] Associations between IgA deficiency and certain major histocompatibility complex (MHC) class I, II, and III haplotypes have been proposed. […] The variation in incidence may also arise from the fact that the definition of selective IgA deficiency may differ in each study or registry.

• #85 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #86 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  Selective IgA deficiency (SIgAD) is the most common human primary immune deficiency (PID). […] Intrinsic defects in the maturation of B cells and a perturbation of Th cells and/or cytokine signals have been hypothesized to contribute to SIgAD pathogenesis. […] The genetic basis of IgA deficiency remains to be clarified. […] Causes of isolated IgA deficiency other than SIgAD include drugs, infections, monogenic diseases and chromosomal abnormalities. […] The considerable variability in clinical manifestations suggests multiple mechanisms contributing to the pathogenesis of SIgAD. […] Intrinsic defects in the class switch recombination (CSR) mechanism and in the maturation of B cells have been reported, with decreased levels of peripheral class-switched memory B cells who cannot differentiate into IgA-secreting plasma cells.

• #87

  https://link.springer.com/article/10.1007/s10875-009-9357-x

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] The defect appears to involve the stem cells since IgA deficiency can be transferred by bone marrow transplantation. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] An intrinsic B cell defect, T helper cell dysfunction, and suppressor T cells have all been reported in IgA deficiency.

• #88 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] It is interesting that IL-21 stimulation has been shown to induce class switch recombination to IgG and IgA and differentiation of IgA and IG secreting plasma cells with restoration of immunoglobulin production ex vivo in patients with IgA deficiency and common variable immunodeficiency (CVID).

• #89 Selective immunoglobulin A deficiency – Wikipedia

  https://en.wikipedia.org/wiki/Selective_immunoglobulin_A_deficiency

  Selective IgA deficiency is inherited in less than half of cases, but has been associated with differences in chromosomes 18, 14 and 6. […] Selective IgA deficiency can be inherited, but fewer than half of all cases but has been associated with some congenital intrauterine infections. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is an inherited inability to produce immunoglobulin A (IgA), a part of the body’s defenses against infection at the body’s surfaces (mainly the surfaces of the respiratory and digestive systems).

• #90 Selective IgA Deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2821513/

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] It is interesting that IL-21 stimulation has been shown to induce class switch recombination to IgG and IgA and differentiation of IgA and IG secreting plasma cells with restoration of immunoglobulin production ex vivo in patients with IgA deficiency and common variable immunodeficiency (CVID).

• #91 Selective IgA deficiency – GPnotebook

  https://gpnotebook.com/pages/infectious-disease/selective-iga-deficiency

  Virtual absence of serum and secretory IgA, and occasionally of IgG2 and IgG4 is relatively common, affecting 1 in 600 people. It may be familial or may be acquired following toxoplasmosis, measles, or other viral infections. […] The basic defect is a failure of maturation of IgA positive B cells.

• #92 IgA deficiency – GPnotebook

  https://gpnotebook.com/en-GB/pages/infectious-disease/iga-deficiency

  Virtual absence of serum and secretory IgA, and occasionally of IgG2 and IgG4 is relatively common, affecting 1 in 600 people. It may be familial or may be acquired following toxoplasmosis, measles, or other viral infections. […] The basic defect is a failure of maturation of IgA positive B cells. Immature forms are present in normal numbers.

• #93

  https://link.springer.com/article/10.1007/s10875-009-9357-x

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] The defect appears to involve the stem cells since IgA deficiency can be transferred by bone marrow transplantation. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] An intrinsic B cell defect, T helper cell dysfunction, and suppressor T cells have all been reported in IgA deficiency.

• #94

  https://link.springer.com/article/10.1007/s10875-009-9357-x

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA in the presence of normal levels of other immunoglobulin isotypes. […] In IgA deficiency, a maturation defect in B cells to produce IgA is commonly observed. […] The genetic basis of IgA deficiency remains to be clarified. […] The defect appears to involve the stem cells since IgA deficiency can be transferred by bone marrow transplantation. […] There is a not well-defined genetic susceptibility in IgA deficiency. […] In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells. […] An intrinsic B cell defect, T helper cell dysfunction, and suppressor T cells have all been reported in IgA deficiency.

• #95 Low IgA and Selective IgA Deficiency

  https://www.geneticlifehacks.com/low-iga-and-selective-deficiency/

  Selective IgA deficiency can be (partly) genetic, or it can be due to other causes such as medications or autoimmunity. […] IgA deficiency is a genetic immunodeficiency disorder characterized by the absence or very low levels of immunoglobulin A (IgA). […] One reason for low IgA is depletion of CD4+CD25+ Treg cells. Another is the inability of B cells to switch classes. Yet another mechanism is reduced proliferative activity of CD4+ T cells. […] IgA deficiency can be inherited, and there is a genetic component to it for most people. […] Certain HLA types are associated with an increased susceptibility to IgA deficiency, and mutations related to common variable immunodeficiency can also cause IgA deficiency. […] Certain bacteria and viruses can secrete an enzyme that destroys IgA, resulting in an IgA deficiency during infection. […] IgA deficiency may also be autoimmune or caused by certain medications.

• #96 Selective IgA Deficiency in Children: Clinical Manifestations, Evaluation, and Management

  https://www.contemporarypediatrics.com/view/selective-iga-deficiency-children-clinical-manifestations-evaluation-and-management

  Selective IgA deficiency (SIGAD) is a genetic disorder with a heterogeneous clinical presentation. In children with SIGAD, B cells expressing surface IgA are present, but they are developmentally arrested. The clinical heterogeneity may be partially reflected in the extent of an arrest in B cell differentiation and, in some cases, an associated cellular immune defect. The inability of the B cells to differentiate into IgA-secreting plasma cells may be caused by the deficiency of cytokines, such as interleukin (IL)-4, IL-6, IL-7, and IL-10.7 […] A mutation in the tumor necrosis factor receptor family member TACI (transmembrane activator and calcium-modulator and cyclophilin ligand interactor) was the first genetic abnormality found in patients with SIGAD. The function of TACI is to facilitate isotype switching in B cells.8 This mutation has also been observed in some patients with CVID, which may explain why some cases of SIGAD can progress to CVID.9 Function-loss mutations in several nonmajor histocompatibility complex (MHC) genes have been found in families with SIGAD or CVID disorders, in which progression may occur from normal to CVID or to SIGAD with or without IgG subclass deficiency.10 Familial grouping of IgA deficiency has also been found; however, the pattern of inheritance has not yet been clearly identified. 11

• #97 Incident — Case 280

  https://path.upmc.edu/cases/case280/inc.html

  The development of IgA deficiency probably resulted then from a failure of these IgA-bearing lymphocytes to differentiate into plasma cells. […] A predisposing locus has been reported in the HLA class II or class III region and a number of abnormalities in the cytokine network are also currently under investigation.

• #98 Selective IgA Deficiency in Children: Clinical Manifestations, Evaluation, and Management

  https://www.contemporarypediatrics.com/view/selective-iga-deficiency-children-clinical-manifestations-evaluation-and-management

  Selective IgA deficiency (SIGAD) is a genetic disorder with a heterogeneous clinical presentation. In children with SIGAD, B cells expressing surface IgA are present, but they are developmentally arrested. The clinical heterogeneity may be partially reflected in the extent of an arrest in B cell differentiation and, in some cases, an associated cellular immune defect. The inability of the B cells to differentiate into IgA-secreting plasma cells may be caused by the deficiency of cytokines, such as interleukin (IL)-4, IL-6, IL-7, and IL-10.7 […] A mutation in the tumor necrosis factor receptor family member TACI (transmembrane activator and calcium-modulator and cyclophilin ligand interactor) was the first genetic abnormality found in patients with SIGAD. The function of TACI is to facilitate isotype switching in B cells.8 This mutation has also been observed in some patients with CVID, which may explain why some cases of SIGAD can progress to CVID.9 Function-loss mutations in several nonmajor histocompatibility complex (MHC) genes have been found in families with SIGAD or CVID disorders, in which progression may occur from normal to CVID or to SIGAD with or without IgG subclass deficiency.10 Familial grouping of IgA deficiency has also been found; however, the pattern of inheritance has not yet been clearly identified. 11

• #99 Immunoglobulin A Deficiency: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/136580-overview

  Evidence for a common pathogenesis of CVID and IgAD include shared susceptibility alleles of major histocompatibility complex class III genes (D locus). […] Previous studies of multiple-case families of patients with IgAD showed a higher prevalence of CVID among close relatives than in the general population. […] IgAD has been reported in patients with constitutional chromosome 18 abnormalities, and a case series of 83 cases of 18p- syndrome showed an increased frequency of IgAD; however, attempts to identify a specific locus on chromosome 18 have not been successful. […] The ability of many patients with SIgAD to avoid respiratory infections may relate to compensatory mechanisms at the respiratory mucosal surface and/or compensatory increases in IgG. […] The TACI receptor and 2 ligands (BAFF and APRIL) likely play a role in the pathogenesis of defective humoral immunity. […] Missense mutations in one allele of TACI were found in 4 of 19 unrelated individuals with CVID and in 1 of 16 individuals with SIgAD.

• #100 Frontiers | Innate Mechanisms in Selective IgA Deficiency

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.649112/full

  SIgAD associations were found with a major predisposing locus on chromosome 18 and structural changes including deletion of the short or the long arm or the presence of a ring-like structure of this chromosome are associated with SIgAD. […] The observation that both SIgAD and CVID can occur in families points towards a shared genetic defect based on shared susceptibility loci and alleles. […] T-cell independent IgA switching is facilitated by the presence of cytokines like BAFF (B cell activating factor) and APRIL binding to their relevant receptors TACI (transmembrane activator and calcium-modulating cyclophilin ligand interaction), BCMA (B cell maturation- antigen) and BAFF-R (BAFF-receptor). […] Defective IgA class switching might still be the main molecular defect in SIgAD.

• #101 Immunoglobulin A Deficiency: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/136580-overview

  Selective immunoglobulin A deficiency (SIgAD) is a primary immunodeficiency disease and is the most common of the primary antibody deficiencies. […] IgAD is a heterogeneous disorder, and the results of intensive study are beginning to elucidate genetic loci and molecular pathogenesis that contribute to various subtypes of this disorder. […] Environmental factors such as drugs or infections can cause IgAD, but this form is reversible in more than half the cases (see Causes). […] Molecular analyses of B-cell differentiation in a small number of patients with selective or partial IgAD suggest that decreased expression level of alpha germline transcripts before a class switch might be critical for the pathogenesis of some patients with SIgAD. […] IgAD has been noted to evolve into CVID and is often observed in pedigrees that contain individuals with CVID.

• #102 Immunoglobulin A Deficiency: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/136580-overview

  Evidence for a common pathogenesis of CVID and IgAD include shared susceptibility alleles of major histocompatibility complex class III genes (D locus). […] Previous studies of multiple-case families of patients with IgAD showed a higher prevalence of CVID among close relatives than in the general population. […] IgAD has been reported in patients with constitutional chromosome 18 abnormalities, and a case series of 83 cases of 18p- syndrome showed an increased frequency of IgAD; however, attempts to identify a specific locus on chromosome 18 have not been successful. […] The ability of many patients with SIgAD to avoid respiratory infections may relate to compensatory mechanisms at the respiratory mucosal surface and/or compensatory increases in IgG. […] The TACI receptor and 2 ligands (BAFF and APRIL) likely play a role in the pathogenesis of defective humoral immunity. […] Missense mutations in one allele of TACI were found in 4 of 19 unrelated individuals with CVID and in 1 of 16 individuals with SIgAD.

• #103 Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-023-00826-y

  The lack of IgA production tend to incite compensatory increase in secretory IgM. […] The spectrum of clinical manifestations in PIgAD and SIgAD are mainly similar with a few minor discrepancies in allergic presentations. […] The coexistence of IgA and IgG subclass deficiency in patients may increase the susceptibility to infections.

• #104 Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-023-00826-y

  The lack of IgA production tend to incite compensatory increase in secretory IgM. […] The spectrum of clinical manifestations in PIgAD and SIgAD are mainly similar with a few minor discrepancies in allergic presentations. […] The coexistence of IgA and IgG subclass deficiency in patients may increase the susceptibility to infections.

• #105 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #106 Immunoglobulin A Deficiency: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/136580-overview

  Evidence for a common pathogenesis of CVID and IgAD include shared susceptibility alleles of major histocompatibility complex class III genes (D locus). […] Previous studies of multiple-case families of patients with IgAD showed a higher prevalence of CVID among close relatives than in the general population. […] IgAD has been reported in patients with constitutional chromosome 18 abnormalities, and a case series of 83 cases of 18p- syndrome showed an increased frequency of IgAD; however, attempts to identify a specific locus on chromosome 18 have not been successful. […] The ability of many patients with SIgAD to avoid respiratory infections may relate to compensatory mechanisms at the respiratory mucosal surface and/or compensatory increases in IgG. […] The TACI receptor and 2 ligands (BAFF and APRIL) likely play a role in the pathogenesis of defective humoral immunity. […] Missense mutations in one allele of TACI were found in 4 of 19 unrelated individuals with CVID and in 1 of 16 individuals with SIgAD.

• #107 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #108 Selective IgA deficiency | Immune Deficiency Foundation

  https://primaryimmune.org/understanding-primary-immunodeficiency/types-of-pi/selective-iga-deficiency

  In those with SIgAD who have an autoimmune disease, a variety of autoantibodies (like ANA or rheumatoid factor) may be positive. […] People with SIgAD (7 mg/dL) are often considered to be at increased risk of life-threatening allergic reactions, also known as an anaphylactic reaction, to blood and blood products, including immunoglobulin (Ig) replacement therapy that may contain traces of IgA.

• #109 Selective Immunoglobulin A (IgA) Deficiency – Immune Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/immune-disorders/immunodeficiency-disorders/selective-immunoglobulin-a-iga-deficiency

  Selective immunoglobulin deficiency is usually inherited. It may be caused by a mutation in a specific gene or by a medication, such as phenytoin (used to treat seizure disorders) or sulfasalazine (used to treat rheumatoid arthritis). The inheritance pattern of the gene mutation is unknown, but having a family member with selective IgA deficiency increases the risk by about 50 times. […] It may be caused by a mutation in a specific gene or by a medication, such as phenytoin (used to treat seizure disorders) or sulfasalazine (used to treat rheumatoid arthritis). […] Some people with selective IgA deficiency produce antibodies against IgA if they are exposed to IgA in blood transfusions or immune globulin (antibodies obtained from the blood of people with a normal immune system). Rarely, these antibodies against IgA trigger a severe allergic (anaphylactic) reaction the next time such people are given a blood transfusion or immune globulin.

• #110 Selective IgA deficiency | Immune Deficiency Foundation

  https://primaryimmune.org/understanding-primary-immunodeficiency/types-of-pi/selective-iga-deficiency

  In those with SIgAD who have an autoimmune disease, a variety of autoantibodies (like ANA or rheumatoid factor) may be positive. […] People with SIgAD (7 mg/dL) are often considered to be at increased risk of life-threatening allergic reactions, also known as an anaphylactic reaction, to blood and blood products, including immunoglobulin (Ig) replacement therapy that may contain traces of IgA.

• #111 Selective Immunoglobulin A (IgA) Deficiency – Immune Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/immune-disorders/immunodeficiency-disorders/selective-immunoglobulin-a-iga-deficiency

  Selective immunoglobulin deficiency is usually inherited. It may be caused by a mutation in a specific gene or by a medication, such as phenytoin (used to treat seizure disorders) or sulfasalazine (used to treat rheumatoid arthritis). The inheritance pattern of the gene mutation is unknown, but having a family member with selective IgA deficiency increases the risk by about 50 times. […] It may be caused by a mutation in a specific gene or by a medication, such as phenytoin (used to treat seizure disorders) or sulfasalazine (used to treat rheumatoid arthritis). […] Some people with selective IgA deficiency produce antibodies against IgA if they are exposed to IgA in blood transfusions or immune globulin (antibodies obtained from the blood of people with a normal immune system). Rarely, these antibodies against IgA trigger a severe allergic (anaphylactic) reaction the next time such people are given a blood transfusion or immune globulin.

• #112 IgA Deficiency – AmeriPharma® Specialty Care

  https://ameripharmaspecialty.com/other-health-conditions/iga-deficiency/

  Some medications also lead to low IgA levels, including: […] IgA deficiency may lead to asthma and allergies. An anaphylactic reaction (severe allergic reaction) is the most common complication. […] IgA deficiency can become serious and can cause a life-threatening condition known as anaphylaxis (a severe allergic reaction). But most of the time, it only increases the susceptibility to infection and sickness. […] Therefore, the treatment of immunoglobulin A deficiency mainly focuses on treating problems associated with IgA deficiency along with some ways to improve IgA levels, but a permanent cure is still not available.

• #113 Selective IgA Deficiency – Immunology; Allergic Disorders – MSD Manual Professional Edition

  https://www.msdmanuals.com/professional/immunology-allergic-disorders/immunodeficiency-disorders/selective-iga-deficiency

  Because immune globulin replacement therapy contains mostly IgG and minimal amounts of IgA, patients with IgA deficiency do not benefit from it. However, there still is some risk of sensitizing patients to IgA or triggering an anaphylactic reaction in those who previously developed anti-IgA antibodies. […] A few patients with IgA deficiency develop CVID over time; others improve spontaneously.

• #114 Selective IgA deficiency | Immune Deficiency Foundation

  https://primaryimmune.org/understanding-primary-immunodeficiency/types-of-pi/selective-iga-deficiency

  In those with SIgAD who have an autoimmune disease, a variety of autoantibodies (like ANA or rheumatoid factor) may be positive. […] People with SIgAD (7 mg/dL) are often considered to be at increased risk of life-threatening allergic reactions, also known as an anaphylactic reaction, to blood and blood products, including immunoglobulin (Ig) replacement therapy that may contain traces of IgA.

• #115 Anaphylactic transfusion reactions and IgA deficiency | Professional Education

  https://professionaleducation.blood.ca/en/transfusion/publications/anaphylactic-transfusion-reactions-and-iga-deficiency

  If the frequency of IgA deficiency and anti-IgA is similar in recipients and in donors, one would expect that approximately 1 to 1.6 patients in 1,000 in Canada would be IgA deficient with anti-IgA. […] However, the frequency of anaphylactic transfusion reactions related to anti-IgA is less than 1 in 500,000 components transfused annually. […] The vast majority of anti-IgA antibodies do not appear to cause anaphylactic transfusion reactions. […] Hence, even in the presence of anti-IgA antibodies in the recipient, risk of severe allergic or anaphylactic transfusion reaction is low.

• #116 Anaphylactic transfusion reactions and IgA deficiency | Professional Education

  https://professionaleducation.blood.ca/en/transfusion/publications/anaphylactic-transfusion-reactions-and-iga-deficiency

  If the frequency of IgA deficiency and anti-IgA is similar in recipients and in donors, one would expect that approximately 1 to 1.6 patients in 1,000 in Canada would be IgA deficient with anti-IgA. […] However, the frequency of anaphylactic transfusion reactions related to anti-IgA is less than 1 in 500,000 components transfused annually. […] The vast majority of anti-IgA antibodies do not appear to cause anaphylactic transfusion reactions. […] Hence, even in the presence of anti-IgA antibodies in the recipient, risk of severe allergic or anaphylactic transfusion reaction is low.

• #117 Selective IgA deficiency | Immune Deficiency Foundation

  https://primaryimmune.org/understanding-primary-immunodeficiency/types-of-pi/selective-iga-deficiency

  In those with SIgAD who have an autoimmune disease, a variety of autoantibodies (like ANA or rheumatoid factor) may be positive. […] People with SIgAD (7 mg/dL) are often considered to be at increased risk of life-threatening allergic reactions, also known as an anaphylactic reaction, to blood and blood products, including immunoglobulin (Ig) replacement therapy that may contain traces of IgA.

• #118 Low IgA & Selective IgA Deficiency: Causes & Symptoms – SelfDecode Labs

  https://labs.selfdecode.com/blog/iga-deficiency/

  Low IgA can be caused by selective IgA deficiency, when only IgA levels are low but other antibodies (IgG, IgM, IgD, and IgE) are normal. […] Selective IgA deficiency can be inherited or occur spontaneously (due to infections, medication, or unknown causes). The genetic disorder that causes people to lack IgA is found in up to 1% of the population. […] People are IgA deficient if they have blood IgA levels below 7 mg/dL with normal IgM and IgG levels. […] Selective IgA deficiency is when IgA levels are 7 mg/dL but IgM and IgG are normal. It can be caused by genetic disorders and non-genetic factors. […] Patients with selective IgA deficiency (undetectable IgA but normal IgG and IgM levels) have a 10 to 20 times higher risk of celiac disease. […] People with IgA deficiency have a higher association of type 1 diabetes (up to 10 times), lupus, juvenile idiopathic arthritis, IBD (both Crohns and ulcerative colitis), rheumatoid arthritis, and thyroid disorders. […] Some studies have associated IgA deficiency with IBD and IBS, though the research is still inconclusive. […] Those with IgA deficiency have a moderately increased risk of cancer, especially gut cancer.

• #119 Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-023-00826-y

  Immunoglobulin A deficiency (IgAD) is a common disease with an unknown genetic defect, characterized by the decreased or absent IgA with other isotypes normal, normal subclasses, and specific antibodies. […] The spectrum of SIgAD clinical manifestations is varied. SIgAD is distinguished from other immunodeficiencies as more than 50% of the affected individuals do not show any clinical symptoms. […] The other major complications in SIgAD, in the order of prevalence, are allergy (29.16%), autoimmunity (22%), and malignancy (3.7%). […] An association between IgAD and a higher prevalence of autoimmune diseases has been detected. […] Celiac disease (6.57%), inflammatory bowel disease (IBD) (4.01%), and rheumatoid arthritis (3.80%) were the most prevalent autoimmunity in SIgAD patients which is much lower than their prevalence in general population.

• #120 Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-023-00826-y

  Immunoglobulin A deficiency (IgAD) is a common disease with an unknown genetic defect, characterized by the decreased or absent IgA with other isotypes normal, normal subclasses, and specific antibodies. […] The spectrum of SIgAD clinical manifestations is varied. SIgAD is distinguished from other immunodeficiencies as more than 50% of the affected individuals do not show any clinical symptoms. […] The other major complications in SIgAD, in the order of prevalence, are allergy (29.16%), autoimmunity (22%), and malignancy (3.7%). […] An association between IgAD and a higher prevalence of autoimmune diseases has been detected. […] Celiac disease (6.57%), inflammatory bowel disease (IBD) (4.01%), and rheumatoid arthritis (3.80%) were the most prevalent autoimmunity in SIgAD patients which is much lower than their prevalence in general population.

• #121 Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-023-00826-y

  Immunoglobulin A deficiency (IgAD) is a common disease with an unknown genetic defect, characterized by the decreased or absent IgA with other isotypes normal, normal subclasses, and specific antibodies. […] The spectrum of SIgAD clinical manifestations is varied. SIgAD is distinguished from other immunodeficiencies as more than 50% of the affected individuals do not show any clinical symptoms. […] The other major complications in SIgAD, in the order of prevalence, are allergy (29.16%), autoimmunity (22%), and malignancy (3.7%). […] An association between IgAD and a higher prevalence of autoimmune diseases has been detected. […] Celiac disease (6.57%), inflammatory bowel disease (IBD) (4.01%), and rheumatoid arthritis (3.80%) were the most prevalent autoimmunity in SIgAD patients which is much lower than their prevalence in general population.

• #122 Low IgA & Selective IgA Deficiency: Causes & Symptoms – SelfDecode Labs

  https://labs.selfdecode.com/blog/iga-deficiency/

  Low IgA can be caused by selective IgA deficiency, when only IgA levels are low but other antibodies (IgG, IgM, IgD, and IgE) are normal. […] Selective IgA deficiency can be inherited or occur spontaneously (due to infections, medication, or unknown causes). The genetic disorder that causes people to lack IgA is found in up to 1% of the population. […] People are IgA deficient if they have blood IgA levels below 7 mg/dL with normal IgM and IgG levels. […] Selective IgA deficiency is when IgA levels are 7 mg/dL but IgM and IgG are normal. It can be caused by genetic disorders and non-genetic factors. […] Patients with selective IgA deficiency (undetectable IgA but normal IgG and IgM levels) have a 10 to 20 times higher risk of celiac disease. […] People with IgA deficiency have a higher association of type 1 diabetes (up to 10 times), lupus, juvenile idiopathic arthritis, IBD (both Crohns and ulcerative colitis), rheumatoid arthritis, and thyroid disorders. […] Some studies have associated IgA deficiency with IBD and IBS, though the research is still inconclusive. […] Those with IgA deficiency have a moderately increased risk of cancer, especially gut cancer.

• #123 IgA Deficiency and Celiac Disease | BeyondCeliac.org

  https://www.beyondceliac.org/celiac-disease/related-conditions/iga-deficiency/

  Selective immunoglobulin A (IgA) deficiency is a genetic immunodeficiency condition in which individuals do not make or have very low blood levels of IgA antibodies. […] It is estimated that 1 in 500 people have selective IgA deficiency. […] For some with IgA deficiency, the condition is ‘silent,’ meaning they have no symptoms, however for others it is associated with a heightened risk of infections, allergies, asthma and autoimmune diseases. […] An estimated 2% of people with celiac disease also have selective immunoglobulin A (IgA) deficiency. […] People with IgA deficiency should be tested for celiac disease because they are 10 to 20 times likely to develop an autoimmune response to gluten than the general population. […] Those with IgA deficiency should be tested for IgG-tTG antibodies instead of IgA-tTG.

• #124 Low IgA & Selective IgA Deficiency: Causes & Symptoms – SelfDecode Labs

  https://labs.selfdecode.com/blog/iga-deficiency/

  Low IgA can be caused by selective IgA deficiency, when only IgA levels are low but other antibodies (IgG, IgM, IgD, and IgE) are normal. […] Selective IgA deficiency can be inherited or occur spontaneously (due to infections, medication, or unknown causes). The genetic disorder that causes people to lack IgA is found in up to 1% of the population. […] People are IgA deficient if they have blood IgA levels below 7 mg/dL with normal IgM and IgG levels. […] Selective IgA deficiency is when IgA levels are 7 mg/dL but IgM and IgG are normal. It can be caused by genetic disorders and non-genetic factors. […] Patients with selective IgA deficiency (undetectable IgA but normal IgG and IgM levels) have a 10 to 20 times higher risk of celiac disease. […] People with IgA deficiency have a higher association of type 1 diabetes (up to 10 times), lupus, juvenile idiopathic arthritis, IBD (both Crohns and ulcerative colitis), rheumatoid arthritis, and thyroid disorders. […] Some studies have associated IgA deficiency with IBD and IBS, though the research is still inconclusive. […] Those with IgA deficiency have a moderately increased risk of cancer, especially gut cancer.

• #125 Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-023-00826-y

  Immunoglobulin A deficiency (IgAD) is a common disease with an unknown genetic defect, characterized by the decreased or absent IgA with other isotypes normal, normal subclasses, and specific antibodies. […] The spectrum of SIgAD clinical manifestations is varied. SIgAD is distinguished from other immunodeficiencies as more than 50% of the affected individuals do not show any clinical symptoms. […] The other major complications in SIgAD, in the order of prevalence, are allergy (29.16%), autoimmunity (22%), and malignancy (3.7%). […] An association between IgAD and a higher prevalence of autoimmune diseases has been detected. […] Celiac disease (6.57%), inflammatory bowel disease (IBD) (4.01%), and rheumatoid arthritis (3.80%) were the most prevalent autoimmunity in SIgAD patients which is much lower than their prevalence in general population.

• #126 Low IgA & Selective IgA Deficiency: Causes & Symptoms – SelfDecode Labs

  https://labs.selfdecode.com/blog/iga-deficiency/

  Low IgA can be caused by selective IgA deficiency, when only IgA levels are low but other antibodies (IgG, IgM, IgD, and IgE) are normal. […] Selective IgA deficiency can be inherited or occur spontaneously (due to infections, medication, or unknown causes). The genetic disorder that causes people to lack IgA is found in up to 1% of the population. […] People are IgA deficient if they have blood IgA levels below 7 mg/dL with normal IgM and IgG levels. […] Selective IgA deficiency is when IgA levels are 7 mg/dL but IgM and IgG are normal. It can be caused by genetic disorders and non-genetic factors. […] Patients with selective IgA deficiency (undetectable IgA but normal IgG and IgM levels) have a 10 to 20 times higher risk of celiac disease. […] People with IgA deficiency have a higher association of type 1 diabetes (up to 10 times), lupus, juvenile idiopathic arthritis, IBD (both Crohns and ulcerative colitis), rheumatoid arthritis, and thyroid disorders. […] Some studies have associated IgA deficiency with IBD and IBS, though the research is still inconclusive. […] Those with IgA deficiency have a moderately increased risk of cancer, especially gut cancer.

• #127 Low IgA & Selective IgA Deficiency: Causes & Symptoms – SelfDecode Labs

  https://labs.selfdecode.com/blog/iga-deficiency/

  Low IgA can be caused by selective IgA deficiency, when only IgA levels are low but other antibodies (IgG, IgM, IgD, and IgE) are normal. […] Selective IgA deficiency can be inherited or occur spontaneously (due to infections, medication, or unknown causes). The genetic disorder that causes people to lack IgA is found in up to 1% of the population. […] People are IgA deficient if they have blood IgA levels below 7 mg/dL with normal IgM and IgG levels. […] Selective IgA deficiency is when IgA levels are 7 mg/dL but IgM and IgG are normal. It can be caused by genetic disorders and non-genetic factors. […] Patients with selective IgA deficiency (undetectable IgA but normal IgG and IgM levels) have a 10 to 20 times higher risk of celiac disease. […] People with IgA deficiency have a higher association of type 1 diabetes (up to 10 times), lupus, juvenile idiopathic arthritis, IBD (both Crohns and ulcerative colitis), rheumatoid arthritis, and thyroid disorders. […] Some studies have associated IgA deficiency with IBD and IBS, though the research is still inconclusive. […] Those with IgA deficiency have a moderately increased risk of cancer, especially gut cancer.

• #128 Low IgA & Selective IgA Deficiency: Causes & Symptoms – SelfDecode Labs

  https://labs.selfdecode.com/blog/iga-deficiency/

  Low IgA can be caused by selective IgA deficiency, when only IgA levels are low but other antibodies (IgG, IgM, IgD, and IgE) are normal. […] Selective IgA deficiency can be inherited or occur spontaneously (due to infections, medication, or unknown causes). The genetic disorder that causes people to lack IgA is found in up to 1% of the population. […] People are IgA deficient if they have blood IgA levels below 7 mg/dL with normal IgM and IgG levels. […] Selective IgA deficiency is when IgA levels are 7 mg/dL but IgM and IgG are normal. It can be caused by genetic disorders and non-genetic factors. […] Patients with selective IgA deficiency (undetectable IgA but normal IgG and IgM levels) have a 10 to 20 times higher risk of celiac disease. […] People with IgA deficiency have a higher association of type 1 diabetes (up to 10 times), lupus, juvenile idiopathic arthritis, IBD (both Crohns and ulcerative colitis), rheumatoid arthritis, and thyroid disorders. […] Some studies have associated IgA deficiency with IBD and IBS, though the research is still inconclusive. […] Those with IgA deficiency have a moderately increased risk of cancer, especially gut cancer.

• #129 Low IgA & Selective IgA Deficiency: Causes & Symptoms – SelfDecode Labs

  https://labs.selfdecode.com/blog/iga-deficiency/

  Low IgA can be caused by selective IgA deficiency, when only IgA levels are low but other antibodies (IgG, IgM, IgD, and IgE) are normal. […] Selective IgA deficiency can be inherited or occur spontaneously (due to infections, medication, or unknown causes). The genetic disorder that causes people to lack IgA is found in up to 1% of the population. […] People are IgA deficient if they have blood IgA levels below 7 mg/dL with normal IgM and IgG levels. […] Selective IgA deficiency is when IgA levels are 7 mg/dL but IgM and IgG are normal. It can be caused by genetic disorders and non-genetic factors. […] Patients with selective IgA deficiency (undetectable IgA but normal IgG and IgM levels) have a 10 to 20 times higher risk of celiac disease. […] People with IgA deficiency have a higher association of type 1 diabetes (up to 10 times), lupus, juvenile idiopathic arthritis, IBD (both Crohns and ulcerative colitis), rheumatoid arthritis, and thyroid disorders. […] Some studies have associated IgA deficiency with IBD and IBS, though the research is still inconclusive. […] Those with IgA deficiency have a moderately increased risk of cancer, especially gut cancer.

• #130 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #131 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #132 Selective IgA Deficiency in Autoimmune Diseases | Molecular Medicine | Full Text

  https://molmed.biomedcentral.com/articles/10.2119/molmed.2011.00195

  Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in Caucasians. Genetic factors are important for the development of both IgAD and various autoimmune disorders, including GD, SLE, T1D, CD, MG and RA, and a strong association with the major histocompatibility complex (MHC) region has been reported. […] In addition, non-MHC genes, such as interferon-induced helicase 1 (IFIH1) and c-type lectin domain family 16, member A (CLEC16A), are also associated with the development of IgAD and some of the above diseases. This indicates a possible common genetic background. […] Interestingly, the ancestral 8.1 haplotype is also reported to be associated with Graves disease (GD), systemic lupus erythematosus (SLE), type 1 diabetes (T1D) and celiac disease (CD).

• #133 Selective IgA Deficiency in Autoimmune Diseases | Molecular Medicine | Full Text

  https://molmed.biomedcentral.com/articles/10.2119/molmed.2011.00195

  It is therefore possible that IgAD and selected autoimmune disorders share some of the predisposing genes, thus explaining the increased prevalence of IgAD in certain patient groups. […] A common genetic background for selected autoimmune disorders, such as GD, SLE, T1D, CD and potentially MG and RA, involving both MHC and non-MHC encoded genes, has previously been suggested. […] The gene(s) involved are primarily located within the MHC region, where the population-attributable risk is strong in T1D, CD and RA and moderate in GD and SLE. […] Our recent preliminary work in IgAD, based on 100 multicase families, suggests a similarly strong MHC-associated risk in these patients. However, owing to strong linkage disequilibrium within the MHC region, the gene(s) involved in disease pathogenesis, with the possible exception of HLA-DQ in CD, have not yet been identified. […] It is well documented that IgAD is strongly associated with the MHC region, in particular, the HLA-B8, DR3, DQ2 haplotype. This haplotype is also associated with GD, SLE, T1D and CD.

• #134 Low IgA and Selective IgA Deficiency

  https://www.geneticlifehacks.com/low-iga-and-selective-deficiency/

  Selective IgA deficiency can be (partly) genetic, or it can be due to other causes such as medications or autoimmunity. […] IgA deficiency is a genetic immunodeficiency disorder characterized by the absence or very low levels of immunoglobulin A (IgA). […] One reason for low IgA is depletion of CD4+CD25+ Treg cells. Another is the inability of B cells to switch classes. Yet another mechanism is reduced proliferative activity of CD4+ T cells. […] IgA deficiency can be inherited, and there is a genetic component to it for most people. […] Certain HLA types are associated with an increased susceptibility to IgA deficiency, and mutations related to common variable immunodeficiency can also cause IgA deficiency. […] Certain bacteria and viruses can secrete an enzyme that destroys IgA, resulting in an IgA deficiency during infection. […] IgA deficiency may also be autoimmune or caused by certain medications.

• #135 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #136 Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-023-00826-y

  Immunoglobulin A deficiency (IgAD) is a common disease with an unknown genetic defect, characterized by the decreased or absent IgA with other isotypes normal, normal subclasses, and specific antibodies. […] The spectrum of SIgAD clinical manifestations is varied. SIgAD is distinguished from other immunodeficiencies as more than 50% of the affected individuals do not show any clinical symptoms. […] The other major complications in SIgAD, in the order of prevalence, are allergy (29.16%), autoimmunity (22%), and malignancy (3.7%). […] An association between IgAD and a higher prevalence of autoimmune diseases has been detected. […] Celiac disease (6.57%), inflammatory bowel disease (IBD) (4.01%), and rheumatoid arthritis (3.80%) were the most prevalent autoimmunity in SIgAD patients which is much lower than their prevalence in general population.

• #137 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #138 Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-023-00826-y

  Immunoglobulin A deficiency (IgAD) is a common disease with an unknown genetic defect, characterized by the decreased or absent IgA with other isotypes normal, normal subclasses, and specific antibodies. […] The spectrum of SIgAD clinical manifestations is varied. SIgAD is distinguished from other immunodeficiencies as more than 50% of the affected individuals do not show any clinical symptoms. […] The other major complications in SIgAD, in the order of prevalence, are allergy (29.16%), autoimmunity (22%), and malignancy (3.7%). […] An association between IgAD and a higher prevalence of autoimmune diseases has been detected. […] Celiac disease (6.57%), inflammatory bowel disease (IBD) (4.01%), and rheumatoid arthritis (3.80%) were the most prevalent autoimmunity in SIgAD patients which is much lower than their prevalence in general population.

• #139 IgA Deficiency – AmeriPharma® Specialty Care

  https://ameripharmaspecialty.com/other-health-conditions/iga-deficiency/

  Some medications also lead to low IgA levels, including: […] IgA deficiency may lead to asthma and allergies. An anaphylactic reaction (severe allergic reaction) is the most common complication. […] IgA deficiency can become serious and can cause a life-threatening condition known as anaphylaxis (a severe allergic reaction). But most of the time, it only increases the susceptibility to infection and sickness. […] Therefore, the treatment of immunoglobulin A deficiency mainly focuses on treating problems associated with IgA deficiency along with some ways to improve IgA levels, but a permanent cure is still not available.

• #140 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #141 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #142 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #143 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #144 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

• #145 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  SIgAD is a heterogeneous disorder with the underlying pathogenetic mechanism remaining largely unexplored. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The loss of mucosal IgA might also facilitate the passage of aeroallergens and food antigens, promoting the development of allergies. […] The absence of IgA in the serum may allow the entry of cross-reactive antigens into the circulation leading to the development of autoimmune reactions. […] It has been postulated that serum IgA helps prevent the circulation of allergens and that secretory IgA plays a protective role on the mucosal surface against allergic disease. […] The majority of patients with deficiency of secretory IgA have substitution with secretory IgM. However, in SIgAD allergic patients, proper mucosal compensation of IgM might not be assured, allowing antigens to pass through the mucosa and predispose one to developing allergy in the gut and respiratory tract.

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