Materiały źródłowe

• #1 Selective IgA Deficiency – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/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. […] By the International Consensus definition, the diagnosis is established in individuals older than four years whose blood levels of IgA are below 7 mg/dl but have normal levels of IgG and IgM. […] Without direct signs or symptoms on presentation, the diagnosis of IgA deficiency may require an initial intuitive approach with a clinical suspicion based on indirect findings. […] The European Society for immunodeficiencies (ESID) new criteria for diagnosing selective IgA deficiency include the following constellation: Undetectable serum IgA levels (below 0.07 g/l, measured by nephelometry), although normal serum levels of IgG and IgM (evaluated by nephelometry at least twice).

• #1 Selective IgA deficiency

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20256724

  A diagnosis of selective IgA deficiency is based on a blood test that measures levels of immunoglobulins in the blood. IgA deficiency can be complete or partial. […] Your healthcare professional may order an immunoglobulin blood test because you have had frequent or repeat illnesses. The test also may be a part of a series of lab tests to diagnose or rule out other conditions.

• #1 Immunoglobulin A Deficiency Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Immunoglobulin A deficiency (IgAD) is defined as an undetectable serum IgA level. In the past, this was usually confirmed with the low-level radial immunodiffusion method (lower limit of detection is 50 mg/mL [5 mg/dL]). However, this test is rarely done in current practice, and results are usually reported as 0.07 g/L or 0.05 g/L. The lower limit of detection differs depending on the sensitivity of the method used. It is usually 0.050.1 g/L for nephelometry, 0.05 g/L (5 mg/dL) for low-level radial immunodiffusion plates, and 0.0016 g/L for hemaglutination inhibition techniques. […] […] Normal serum levels of IgG and IgM are necessary for a diagnosis of selective immunoglobulin A deficiency (SIgAD). Other causes of hypogammaglobulinemia and use of medications that reduce IgA levels should be excluded (see Differentials). A total IgG level within the normal range does not exclude specific IgG antibody deficiency (eg, deficient antibodies to polysaccharides), and this should be specifically sought in patients who have recurrent infections, total IgG levels toward the lower bound of normal, and IgA deficiency. Tests showing low IgA serum values in children younger than 5 years should be repeated. Some children with low levels progress to CVID, but levels can normalize by age 4-5 years. […] […] The most common mistake clinicians make with this diagnosis is incorrectly diagnosing IgAD or transient hypogammaglobulinemia of infancy in children because they inappropriately use the adult reference range for serum IgA levels. […]

• #1

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

  Immunoglobulin (Ig) A deficiency (OMIM 137100) is defined as decreased or absent level 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. […] The diagnosis of IgA deficiency depends on the measurement of IgA concentration in serum. Secretory IgA level is not determined; therefore, it is possible that the individuals diagnosed with selective IgA deficiency may still have some IgA in the mucosal systems enough to provide some protective functions. […] IgA deficiency should be a consideration in a patient with recurrent respiratory and gastrointestinal infections, allergies, and autoimmune disorders. […] Immunologic evaluation for IgA deficiency is also warranted in case of anaphylaxis secondary to a blood product transfusion, celiac disease, and a family history of IgA deficiency and/or CVID.

• #1 Selective Immunoglobulin A (IgA) Deficiency – Immune Disorders – MSD Manual Consumer Version

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

  Most people with selective IgA deficiency have few or no symptoms, but some have chronic lung infections, sinusitis, and other disorders. […] Doctors diagnose the disorder by measuring levels of immunoglobulins in the blood. […] Blood tests to measure immunoglobulin levels are done to confirm the diagnosis. […] Doctors measure IgA antibody (immunoglobulin) levels before and after vaccinations. Lack of increase in IgA after vaccination helps to make the diagnosis.

• #1 Selective IgA deficiency | Immune Deficiency Foundation

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

  The diagnosis of SIgAD is usually suspected because of chronic or recurrent ear infections, sinusitis, respiratory tract infections, chronic diarrhea, or some combination of these problems. […] The diagnosis is established when blood tests demonstrate undetectable levels of IgA (reported usually as 7 mg/dL), with normal levels of the other major classes of immunoglobulins (IgG and IgM). […] The healthcare provider may order several other tests including autoantibodies.

• #1 Selective IGA Deficiency

  https://my.clevelandclinic.org/health/diseases/17887-selective-iga-deficiency-

  Most people with selective IgA deficiency do not have any symptoms. Doctors often find the disorder when testing for another condition. […] Immunologists, or doctors who specialize in the immune system, may send blood tests to evaluate a patient who with frequent infections or autoimmune disease. Low levels of IgA confirm the diagnosis of selective IgA deficiency. In many cases, the condition is identified when a patient undergoes blood testing for another condition, like celiac disease, and low levels of IgA are incidentally noted.

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

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

  Selective IgA deficiency should be a possibility in every workup of an autoimmune disease. […] The diagnosis of selective IgA deficiency is often is one of exclusion. The following conditions are examples of what must be excluded while making a diagnosis of IgA deficiency: Common variable immunodeficiency (CVID), where both IgG and IgA decline significantly, although IgM is also reduced in about half the patients. […] With its lack of direct signs and symptoms, IgA deficiency can be overshadowed by other ailments. The presence of clinical acumen, supplemented by suspicion, might be the only and best diagnostic weapon.

• #1 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 the most common immunodeficiency disorder; it affects about 1 in 200 to 900 persons. Most affected children are asymptomatic. […] Consider an evaluation for IgA deficiency in children older than 6 months who have recurrent bacterial infections in the upper or lower respiratory tract. This evaluation includes a complete blood cell count and measurement of peripheral blood B cells, T cells, NK cells, and levels of serum immunoglobulins. […] The diagnosis of SIGAD depends on the serum level of IgA, which is compared with that of age-matched controls. […] Partial IgA deficiency is defined as having a serum IgA level more than 2 standard deviations below the mean for age but above 7 mg/dL. […] Low serum IgA levels in a child younger than 4 years may be the result of a transient form of IgA deficiency. After 4 years of age, however, low or absent serum IgA levels are usually considered to be the result of true IgA deficiency.

• #1 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. […] Diagnosis can be established through a blood test measuring immunoglobulin levels in blood serum. […] An estimated 2% of people with celiac disease also have selective immunoglobulin A (IgA) deficiency. […] If someone has IgA deficiency and celiac disease, the IgA deficiency can cause a false negative on a celiac disease antibody test. […] 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. This is because the IgA-tTG test will not be accurate in those with IgA deficiency.

• #1 Serologic testing for celiac disease in patients with IgA deficiency – Insights

  https://news.mayocliniclabs.com/2021/03/11/serologic-testing-for-celiac-disease-in-patients-with-iga-deficiency/

  Today I’d like to talk about serologic testing for celiac disease in patients with IgA deficiency. […] The approach to testing for celiac disease depends on the pretest probability of the patient and his/her likelihood of having celiac disease. […] If the patient has moderate to high probability based on genetic predisposing factors, or based on the presence of highly characteristic symptoms, then that patient would undergo a serologic screen with either tissue transglutaminase IgA antibodies or antiendomysial or antigliadin IgA antibodies, as well as a total IgA level. The purpose of the total IgA level is to make sure the patient isn’t IgA deficient. […] One of the most important reasons for a falsely negative test, and one we will highlight today with today’s “Hot Topic” presentation, is IgA deficiency.

• #1 Celiac Disease and Iga Deficiency – About a Clinical Case

  https://www.heraldopenaccess.us/openaccess/celiac-disease-and-iga-deficiency-about-a-clinical-case

  The main question and difficulty are knowing how to monitor these patients, that is, how to monitor response to the gluten-free diet (GFD). […] The procedures for diagnosing and monitoring CD in patients with SIgAD still require careful review. […] The only established consensus seems to be the determination of anti-tTG IgA and total serum IgA as an initial screening for all patients with suspected CD. […] Once the presence of SIgAD has been demonstrated, the choice of an IgG-based test necessary to continue the diagnostic investigation remains open. […] There is no strong evidence on which should be the most sensitive and useful IgG marker for both diagnosis and follow-up, given the great heterogeneity of IgG serology in this field. […] Even so, some studies have identified anti-tTG IgG as a more reliable marker for monitoring than anti-DGP IgG for CD in patients with SIgAD, which is the opposite of what we verified with this case.

• #1 Selective IgA Deficiency – StatPearls – NCBI Bookshelf

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

  The diagnosis of selective IgA deficiency is often is one of exclusion. […] Selective IgA deficiency should be a possibility in every workup of an autoimmune disease. […] The prognosis, overall, is typically good. […] The most common complications are associated with respiratory infections and malignancies. […] Selective IgA deficiency is a disorder that requires an interprofessional healthcare team to provide the best diagnostic process, management, and patient follow-up.

• #1 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. It has previously been suggested to be associated with a variety of concomitant autoimmune diseases. […] Most individuals with IgAD are clinically asymptomatic, but the defect may be associated with recurrent respiratory and gastrointestinal tract infections/disorders, autoimmunity and allergies. Patients with IgAD are usually more prone to infections when concomitant IgG subclass deficiency is present. […] An association between GD and IgAD was previously suggested in various case reports, and a small screening study showed that IgAD is overrepresented among GD patients, although this could not be confirmed by other groups. […] Previous studies have reported an increased frequency of IgAD among SLE patients, ranging from 1:19 in the USA to 1:130 in Spain.

• #1 Primary Immunodeficiency Diseases – Immunoglobulin Disorders | Choose the Right Test

  https://arupconsult.com/content/immunoglobulin-disorders

  A careful, stepwise laboratory evaluation is recommended to diagnose an underlying PIDD. […] Immunoglobulin deficiency can be detected by determining total IgG, IgA, and IgM levels and is generally the first step in the workup of immunoglobulin disorders. […] IgA deficiency is defined as decreased or absent IgA in the presence of normal IgG and IgM in a patient older than 4 years in whom other causes of hypogammaglobulinemia have been excluded. […] SIGAD is defined as serum IgA 7 mg/dL (generally the lowest detectable limit established by most laboratories). […] SIGAD is considered the most common PIDD. […] Prognosis is good for individuals with IgA deficiency if it is not associated with a significant disease, and the deficiency may even resolve over time in children. […] However, IgA deficiency can develop into CVID later in life, so monitoring of clinical and immunologic findings is critical.

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

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

  Patients with SIGAD have normal serum levels of IgG, IgM, and IgE and normal cell-mediated immunity. […] Because SIGAD has been found in association with CVID, IgG subclass deficiencies, and impairment of specific antibody responses, the evaluation should not stop with the finding of isolated IgA deficiency. […] Recurrent upper and lower respiratory tract infections from encapsulated bacteria in children who are older than 6 months should prompt an evaluation for possible IgA deficiency.

• #1

  https://www.nicklauschildrens.org/symptoms/iga-deficiency

  Immunoglobulin A, or IgA, is an antibody that is found primarily within the tissues of the body. […] IgA Deficiency is the most common immunodeficiency, and a majority of people who have this are asymptomatic. […] You should be evaluated by a board certified Allergist/Immunologist to determine if you need to take special precautions with blood transfusions. […] You should see a board certified Allergist/Immunologist if you have been found to have low or absent IgA on blood work, if you have recurrent sinopulmonary infections, giardia infection, celiac disease, family history of IgA deficiency, or an anaphylactic reaction to a blood transfusion.

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

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

  Allergy is a quite common clinical complication affecting more than 30% of patients. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The suspicion of SIgAD should heighten not only in patients with recurrent infections but also in those with atopic manifestations. […] Studies analyzing allergy on SIgAD patients showed prevalence ranging from 13 to 84%, supporting in most of the cases the relationship between SIgAD and allergic diseases. […] It is worth mentioning that in many cases allergy is the first symptom of SIgAD, and, in some cases, it is the only symptom of disease. […] The association between SIgAD and allergy was found for various allergic manifestations, the most commonly described of which are asthma, allergic rhinitis, allergic conjunctivitis, urticaria, atopic dermatitis and food allergy, even if the exact prevalence of each symptom and of one clinical manifestation or another varies among the studies reported. […] In light of the association between allergy/asthma and recurrent infections in SIgAD, early detection and management of respiratory disorders is essential to prevent severe complications.

• #1 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 pooled prevalence of infection (in 1,056 IgAD patients), autoimmunity (in 1,660 IgAD patients), at least one allergic disease (in 1,429 IgAD patients), and malignancy (in 904 IgAD patients) was 64.0%, 21.9%, 29.0%, and 3.5%, respectively. In this regard, celiac disease, respiratory tract infection, and asthma were the most prevalence presentation in the group of autoimmunity, infection, and allergic diseases, respectively. […] The prevalence of at least one infection was reported in 13 distinct studies and varied from 12.04% by Petty R.E, et al. from Canada to 98.41% by Mohammadinejad P, et al., from Iran. The pooled prevalence of infection in the study population was 64.8%. Eleven types of infectious presentation were reported by the current study investigations. Based on the meta-analysis result, respiratory tract infection, fungal infection, and gastrointestinal infection were the most frequent infection in SIgAD patients.

• #2 Selective IgA deficiency: Management and prognosis – UpToDate

  https://www.uptodate.com/contents/selective-iga-deficiency-management-and-prognosis

  Selective immunoglobulin A (IgA) deficiency (sIgAD; MIM 137100) is defined as the isolated deficiency of serum IgA (ie, serum levels of immunoglobulin G [IgG] and immunoglobulin M [IgM] are normal) in a patient older than four years of age in whom other causes of hypogammaglobulinemia have been excluded. It is the most common primary antibody defect in humans. […] The clinical manifestations of sIgAD range from an asymptomatic laboratory finding to recurrent infections and autoimmune disease. This topic reviews the management and prognosis of patients with sIgAD. The clinical features, pathophysiology, and diagnosis are discussed separately. (See „Selective IgA deficiency: Clinical manifestations, pathophysiology, and diagnosis”.)

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

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

  Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency characterized by a decreased amount of serum IgA and a concomitant lack of secretory IgA. By the International Consensus definition, the diagnosis is established in individuals older than four years whose blood levels of IgA are below 7 mg/dl but have normal levels of IgG and IgM. Patients who suffer from selective IgA deficiency may have clinical symptoms, but most are largely asymptomatic. […] Without direct signs or symptoms on presentation, the diagnosis of IgA deficiency may require an initial intuitive approach with a clinical suspicion based on indirect findings. […] The European Society for immunodeficiencies (ESID) new criteria for diagnosing selective IgA deficiency include the following constellation: Undetectable serum IgA levels (below 0.07 g/l, measured by nephelometry), although normal serum levels of IgG and IgM (evaluated by nephelometry at least twice).

• #2 Selective IgA Deficiency | Concise Medical Knowledge

  https://www.lecturio.com/concepts/selective-iga-deficiency/

  Diagnosis is made with a measure of exceptionally low IgA levels in the serum in the presence of normal IgG and IgM levels. […] Confirmed by laboratory measurement of IgA 7 mg/dL with normal serum IgG and IgM (measured at least twice) in a patient 4 years of age. […] Secondary causes of hypogammaglobulinemia must be excluded, with a normal IgG antibody response to all vaccinations, and exclusion of a T-cell defect.

• #2 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

  Doctors diagnose the disorder by measuring levels of immunoglobulins in the blood. […] Blood tests to measure immunoglobulin levels are done to confirm the diagnosis. […] Doctors measure IgA antibody (immunoglobulin) levels before and after vaccinations. Lack of increase in IgA after vaccination helps to make the diagnosis.

• #2 IgA deficiency – MyBioSource Learning Center

  https://www.mybiosource.com/learn/iga-deficiency/

  When an individual is presented with the recurrent infections such as chronic diarrhea or allergies, they may then be suspected of this deficiency. The diagnosis is based on the identification of the low levels of IgA in the blood and the saliva but has normal levels of other types of immunoglobulin. The serum immunoglobulin concentrations are measured by the automated laser nephelometry or radioimmunoassay.

• #2

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

  Evaluation of a suspected IgA deficiency would generally include a complete blood count with differential, quantitative serum immunoglobulin levels, serum IgG subclasses, specific antibody response to protein and polysaccharide antigens, and lymphocyte subsets. […] In IgA deficiency, the mainstay of treatment is the treatment of associated diseases.

• #2 Selective IgA Deficiency – Immunology; Allergic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.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. […] Diagnosis is by measuring serum immunoglobulins. […] 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. […] Patients with suspected IgA deficiency should have immunoglobulin levels measured; diagnosis is confirmed by a serum IgA level 7 mg/dL (1). […] Confirm the diagnosis by measuring immunoglobulin levels and antibody titers after vaccines are given; an IgA level.

• #2 Decoding antibody deficiency diagnoses | Immune Deficiency Foundation

  https://primaryimmune.org/resources/news-articles/decoding-antibody-deficiency-diagnoses

  The classic symptom of an antibody deficiency is frequent, severe, and/or recurring infections, including pneumonia, sinus infections, gastroenteritis, skin infections, and sepsis. […] For example, there are people who have both selective IgA deficiency and IgG subclass deficiency. […] Selective IgA deficiency is when someone has little to no IgA but normal levels of IgG and IgM. These individuals essentially lack antibody protection at mucosal barriers where germs get in to the body, but they still have protection from IgG in their bloodstreams. […] Most people with selective IgA deficiency do not have symptoms or have symptoms so mild that they dont seek a diagnosis. Some do have recurrent sinus, respiratory, or gastrointestinal infections. […] Those with symptoms are more likely to go on to develop CVID. […] IgA deficiency correlates with several autoimmune conditions and allergies, especially celiac disease.

• #2 Selective IgA deficiency: Management and prognosis – UpToDate

  https://www.uptodate.com/contents/selective-iga-deficiency-management-and-prognosis/print

  Selective immunoglobulin A (IgA) deficiency (sIgAD; MIM 137100) is defined as the isolated deficiency of serum IgA (ie, serum levels of immunoglobulin G [IgG] and immunoglobulin M [IgM] are normal) in a patient older than four years of age in whom other causes of hypogammaglobulinemia have been excluded. It is the most common primary antibody defect in humans. […] The clinical features, pathophysiology, and diagnosis are discussed separately. (See „Selective IgA deficiency: Clinical manifestations, pathophysiology, and diagnosis”.)

• #2 Quantitative serum immunoglobulin tests

  https://www.racgp.org.au/afp/2013/april/quantitative-serum-immunoglobulin-tests

  Serum immunoglobulin tests are used for the evaluation of antibody (humoral) immunodeficiencies. […] There are a large number of primary humoral immunodeficiencies in which immunoglobulins show a mild or profound alteration (usually decreased, sometimes increased). […] Selective IgA deficiency is one of the most common immunodeficiencies and occurs with a frequency of 1:500. […] Laboratory testing shows undetectable IgA, and normal IgG and IgM. […] This finding is often found incidentally, such as when testing for coeliac disease. […] As serum levels of IgA do not reach adult levels until the age of 8 years, a diagnosis of IgA deficiency should not be made in children less than 4 years of age.

• #2 Celiac Disease and Iga Deficiency – About a Clinical Case

  https://www.heraldopenaccess.us/openaccess/celiac-disease-and-iga-deficiency-about-a-clinical-case

  Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency related to Celiac Disease (CD). […] what is still not definitively known is how to make the diagnosis and monitor patients with both pathologies. […] According to the 2012 ESPGHAN guidelines, the diagnostic criteria for CD are based on the following: symptomatic children and adolescents presenting antitissue transglutaminase (anti-tTG) serum levels over 10 times the upper limit of normal, confirmed by endomysium antibody (EMA) detection and HLA typing in subsequent blood samples, can be diagnosed as having CD without performing duodenal biopsy. […] The gold standard for CD diagnosis is represented by the combination of mucosal changes, namely atrophy (detected by duodenal biopsy) and by positivity of serological tests.

• #2 Serologic testing for celiac disease in patients with IgA deficiency – Insights

  https://news.mayocliniclabs.com/2021/03/11/serologic-testing-for-celiac-disease-in-patients-with-iga-deficiency/

  Patients with celiac disease are substantially more likely than the general population to have IgA deficiency. When a patient is known to have IgA deficiency, then IgG testing should be pursued. […] The most important reason to order these studies would be to optimize diagnostic sensitivity in patients suspected of having celiac disease but with negative IgA testing. In addition, it can be used to establish a diagnosis of celiac disease in a patient known to be IgA deficient. […] In summary, celiac disease is mediated by tissue transglutaminase antibodies, typically of the IgA isoform. However, patients with celiac disease are at higher risk of having IgA deficiency. In this setting, IgA-based testing for celiac disease is often negative, complicating the clinical picture. In that setting, we recommend IgG antibody testing, either against gliadin, tissue transglutaminase, or endomysium, to establish the diagnosis of celiac disease.

• #2 Celiac Disease and Iga Deficiency – About a Clinical Case

  https://www.heraldopenaccess.us/openaccess/celiac-disease-and-iga-deficiency-about-a-clinical-case

  However, we have to bear in mind the possibility of anti-tTG IgG levels remaining high even in a patient who adheres to a GFD, and as such, this antibody does not constitute a very reliable method of monitoring these patients (as regards whether or not they comply with the therapy). […] More studies will be necessary and useful to understand the time of disappearance of specific IgG antibodies, as well as to evaluate the long-term implications of their persistence in the bloodstream; thus, they will help to improve the monitoring of IgA-deficient CD patients undergoing GFD treatment. […] Finally, a serum marker capable of identifying intestinal damage would be desirable as a useful additional tool to fully monitor CD without subjecting patients to multiple duodenal biopsies.

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

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

  Selective IgA deficiency should be a possibility in every workup of an autoimmune disease. […] The diagnosis of selective IgA deficiency is often is one of exclusion. The following conditions are examples of what must be excluded while making a diagnosis of IgA deficiency: Common variable immunodeficiency (CVID), where both IgG and IgA decline significantly, although IgM is also reduced in about half the patients. […] With its lack of direct signs and symptoms, IgA deficiency can be overshadowed by other ailments. The presence of clinical acumen, supplemented by suspicion, might be the only and best diagnostic weapon.

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

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

  The prevalence of IgAD in T1D has been reported to range from 1:27 to 1:261 in several reports, although no cases were observed in three studies, indicating an increased frequency compared to the general population. […] IgAD was previously shown to be associated with CD, with a reported overall prevalence of 1:39, indicating a 5- to 15-fold increase in the prevalence of IgAD among both children and adults with CD. […] Studies on the frequency of IgAD in cohorts of patients with MG are presented, showing that the prevalence of IgAD is slightly elevated from the background rate. […] Studies on RA and JRA contain a total of 5,724 individuals, of whom 74 were IgAD, indicating an increase in IgAD in rheumatic diseases. […] In this report, we have added IgAD to this group of diseases, since it shows a markedly increased prevalence in GD, SLE, T1D and CD.

• #2 Demographic Features, Clinical, and Laboratory Findings of Partial and Selective IgA Deficiency in Children – The Journal of Pediatric Research

  https://jpedres.org/articles/demographic-features-clinical-and-laboratory-findings-of-partial-and-selective-iga-deficiency-in-children/doi/jpr.galenos.2024.89137

  Following an IgAD diagnosis, patients should undergo close immunological and clinical monitoring. […] Our study observed that some patients diagnosed with partial IgAD could switch to selective IgAD, while others in the selective group could switch to partial deficiency. […] It is important to monitor these transitions and the potential for reversals in the risk assessment and follow-up of these patients. […] This study observed that the patients complaints could begin over a wide age range, and most applied to a physician before 4 years of age. […] The most significant risk factor for the development of IgAD and CVID is a positive family history and it is recommended that the families of IgAD and CVID patients undergo routine screening. […] Our results demonstrate that both selective and partial IgAD patients were mostly struggling with recurrent infections, as previously reported.

• #2 Demographic Features, Clinical, and Laboratory Findings of Partial and Selective IgA Deficiency in Children – The Journal of Pediatric Research

  https://jpedres.org/articles/demographic-features-clinical-and-laboratory-findings-of-partial-and-selective-iga-deficiency-in-children/doi/jpr.galenos.2024.89137

  A substantial number of patients demonstrated reversals in a comprehensive study conducted in Sweden, despite the fact that a diagnosis of IgAD was made after the age of 4 years. […] The association between IgAD and autoimmunity may be explained by the relationship between IgAD and abnormal T-cell regulation, particularly in regulatory T-cells and reduced switching memory B-cells. […] Autoimmunity was present in 14.1% of the participants in our investigation. […] The most prevalent autoimmune condition among our patients was autoimmune thyroiditis, which affected 6.0% of individuals. […] This highlights the high frequency of allergy in IgAD patients and the necessity of vigilant monitoring of these individuals for allergic manifestations during both the diagnosis and follow-up phases. […] Ultimately, patients diagnosed with IgAD should undergo extensive immunological and clinical monitoring during their follow-up.

• #2 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 pooled prevalence of infection (in 1,056 IgAD patients), autoimmunity (in 1,660 IgAD patients), at least one allergic disease (in 1,429 IgAD patients), and malignancy (in 904 IgAD patients) was 64.0%, 21.9%, 29.0%, and 3.5%, respectively. In this regard, celiac disease, respiratory tract infection, and asthma were the most prevalence presentation in the group of autoimmunity, infection, and allergic diseases, respectively. […] The prevalence of at least one infection was reported in 13 distinct studies and varied from 12.04% by Petty R.E, et al. from Canada to 98.41% by Mohammadinejad P, et al., from Iran. The pooled prevalence of infection in the study population was 64.8%. Eleven types of infectious presentation were reported by the current study investigations. Based on the meta-analysis result, respiratory tract infection, fungal infection, and gastrointestinal infection were the most frequent infection in SIgAD patients.

• #2 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 prevalence of at least one autoimmunity in SIgAD patients was assessed in 18 studies and ranged from 4.2% by stergaard P.A. from Denmark to 39.2% by Kanoh T., et al. from Japan. The pooled prevalence of autoimmunity in the study populations was 22.0%. Eighteen types of autoimmune manifestations were reported by the current study investigations. Based on meta-analysis results, celiac disease, inflammatory bowel disease (IBD), and rheumatoid arthritis were the most prevalent autoimmunity in SIgAD patients, respectively. […] The prevalence of at least one allergic disease was determined in 16 different studies and fluctuated from 5.50% by Junwu Zhang, et al. from China to 68.25% by Mohammadinejad P, et al., from Iran. The pooled prevalence of at least one allergic disease in the study population was 29.16%. Eight types of allergic diseases were reported by the current study investigations. Based on meta-analysis results, the pooled prevalence of asthma, allergic rhinitis, and allergic conjunctivitis were reported as the most widespread allergic diseases in SIgAD patients.

• #2 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). It is classified as a humoral PID characterized by isolated deficiency of IgA (less than 7 mg/dL but normal serum IgG and IgM) in subjects greater than 4 years of age. […] The aim of this review is to examine the association between SIgAD and atopic disease and to update readers on advances over time at this important interface between allergy and SIgAD. […] The criteria for the definitive diagnosis of SIgAD include patients greater than 4 years of age who have serum IgA levels of less than 7 mg/dL (0.07 g/L) but normal serum IgG and IgM (also defined as isolated IgA deficiency), where other causes of hypogammaglobulinemia have been excluded, and there is normal IgG antibody response to vaccination.

• #2 Selective IgA Deficiency – StatPearls – NCBI Bookshelf

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

  The diagnosis of selective IgA deficiency is often is one of exclusion. […] Selective IgA deficiency should be a possibility in every workup of an autoimmune disease. […] The prognosis, overall, is typically good. […] The most common complications are associated with respiratory infections and malignancies. […] Selective IgA deficiency is a disorder that requires an interprofessional healthcare team to provide the best diagnostic process, management, and patient follow-up.

• #2 Frontiers | Innate Mechanisms in Selective IgA Deficiency

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

  The diagnostic procedure should include the measurement of serum antibodies of IgM, IgG and IgA class and a complete blood cell count complemented with the measurement of peripheral blood B cells, T cells, and NK cells. […] The diagnosis of SIgAD is therefore often accidental when patients are screened for the presence of other diseases like allergies or autoimmune diseases. […] The SIgAD diagnosis is usually based on the measurements of IgA concentrations in the blood of a patient older than four years being below 0.05 mg/ml (5 mg/dL) while levels of IgG and IgM are normal. […] Therefore, in children aged 6 months to 4 years with low serum IgA levels these measurements should be repeated at 4 years of age to confirm the formal diagnosis of SIgAD. […] Therefore, there is a pressing need to design a diagnostic procedure to rapidly identify these children at younger age and to relieve them of the burden of recurrent infections and the predisposition to develop other associated diseases later in life.

• #3 Selective immunoglobulin A deficiency – Wikipedia

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

  Selective immunoglobulin A (IgA) deficiency (SIgAD) is a kind of immunodeficiency, a type of hypogammaglobulinemia. […] It is defined as an undetectable serum IgA level in the presence of normal serum levels of IgG and IgM, in persons older than 4 years. […] When suspected, the diagnosis can be confirmed by laboratory measurement of IgA level in the blood. SIgAD is an IgA level 7 mg/dL with normal IgG and IgM levels (reference range 70400 mg/dL for adults; children somewhat less).

• #3 Selective IgA deficiency | Immune Deficiency Foundation

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

  The diagnosis of SIgAD is usually suspected because of chronic or recurrent ear infections, sinusitis, respiratory tract infections, chronic diarrhea, or some combination of these problems. […] The diagnosis is established when blood tests demonstrate undetectable levels of IgA (reported usually as 7 mg/dL), with normal levels of the other major classes of immunoglobulins (IgG and IgM). […] The healthcare provider may order several other tests including autoantibodies.

• #3 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 the most common immunodeficiency disorder; it affects about 1 in 200 to 900 persons. Most affected children are asymptomatic. […] Consider an evaluation for IgA deficiency in children older than 6 months who have recurrent bacterial infections in the upper or lower respiratory tract. This evaluation includes a complete blood cell count and measurement of peripheral blood B cells, T cells, NK cells, and levels of serum immunoglobulins. […] The diagnosis of SIGAD depends on the serum level of IgA, which is compared with that of age-matched controls. […] Partial IgA deficiency is defined as having a serum IgA level more than 2 standard deviations below the mean for age but above 7 mg/dL. […] Low serum IgA levels in a child younger than 4 years may be the result of a transient form of IgA deficiency. After 4 years of age, however, low or absent serum IgA levels are usually considered to be the result of true IgA deficiency.

• #3 Immunoglobulin A Deficiency Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Immunoglobulin A deficiency (IgAD) is defined as an undetectable serum IgA level. In the past, this was usually confirmed with the low-level radial immunodiffusion method (lower limit of detection is 50 mg/mL [5 mg/dL]). However, this test is rarely done in current practice, and results are usually reported as 0.07 g/L or 0.05 g/L. The lower limit of detection differs depending on the sensitivity of the method used. It is usually 0.050.1 g/L for nephelometry, 0.05 g/L (5 mg/dL) for low-level radial immunodiffusion plates, and 0.0016 g/L for hemaglutination inhibition techniques. […] […] Normal serum levels of IgG and IgM are necessary for a diagnosis of selective immunoglobulin A deficiency (SIgAD). Other causes of hypogammaglobulinemia and use of medications that reduce IgA levels should be excluded (see Differentials). A total IgG level within the normal range does not exclude specific IgG antibody deficiency (eg, deficient antibodies to polysaccharides), and this should be specifically sought in patients who have recurrent infections, total IgG levels toward the lower bound of normal, and IgA deficiency. Tests showing low IgA serum values in children younger than 5 years should be repeated. Some children with low levels progress to CVID, but levels can normalize by age 4-5 years. […] […] The most common mistake clinicians make with this diagnosis is incorrectly diagnosing IgAD or transient hypogammaglobulinemia of infancy in children because they inappropriately use the adult reference range for serum IgA levels. […]

• #3 Selective IgA deficiency

  https://dermnetnz.org/topics/selective-iga-deficiency

  Patients with selective IgA deficiency have normal levels of all immunoglobulins except for IgA. […] Diagnosis of selective IgA deficiency is based on: Patient symptoms, including a history of recurrent infections, chronic diarrhoea and autoimmune diseases […] Quantitative immunoglobulin studies, which generally reveal low IgA and normal levels of other immunoglobulins (though some patients may also have low IgG levels) […] Additional testing, which may include a complete blood count, thyroid, lung and kidney function tests, urinalysis, tests to determine the presence of autoantibodies and nutrient absorption tests.

• #3 Selective IgA Deficiency: Symptoms and Diagnosis Info

  https://myigsource.com/selective-iga-deficiency

  Ongoing or recurrent infections, allergies, sinusitis, respiratory tract infections, chronic diarrhea, or a combination of these issues can lead doctors to suspect selective IgA deficiency in children and adults. […] The diagnosis is made when a blood test shows very low IgA with normal levels of the other major types of immunoglobulins (IgG and IgM).

• #3 Selective deficiency of IgA: MedlinePlus Medical EncyclopediaLock

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

  Selective deficiency of IgA is the most common immune deficiency disorder. People with this disorder have a low or absent level of a blood protein called immunoglobulin A. […] 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. […] There may be a family history of IgA deficiency. Tests that may be done include: IgG subclass measurements, Quantitative immunoglobulins, Serum immunoelectrophoresis. […] People with IgA deficiency should contact their health care provider if they are having symptoms of an infection or intestinal symptoms.

• #3 Frontiers | Innate Mechanisms in Selective IgA Deficiency

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

  The diagnostic procedure should include the measurement of serum antibodies of IgM, IgG and IgA class and a complete blood cell count complemented with the measurement of peripheral blood B cells, T cells, and NK cells. […] The diagnosis of SIgAD is therefore often accidental when patients are screened for the presence of other diseases like allergies or autoimmune diseases. […] The SIgAD diagnosis is usually based on the measurements of IgA concentrations in the blood of a patient older than four years being below 0.05 mg/ml (5 mg/dL) while levels of IgG and IgM are normal. […] Therefore, in children aged 6 months to 4 years with low serum IgA levels these measurements should be repeated at 4 years of age to confirm the formal diagnosis of SIgAD. […] Therefore, there is a pressing need to design a diagnostic procedure to rapidly identify these children at younger age and to relieve them of the burden of recurrent infections and the predisposition to develop other associated diseases later in life.

• #3 Celiac Disease Screening | Celiac Disease FoundationBack to HomeBack to HomeAbout the DiseaseGluten-Free ResourcesResearch ResourcesAdvocacy and Public PolicyJoin the EffortSupport the FoundationShare on FacebookShare on TwitterEmail this pageprinter-mini

  https://celiac.org/about-celiac-disease/screening-and-diagnosis/screening/

  A simple blood test is available to test for celiac disease. People with celiac disease who eat gluten have higher than normal levels of certain antibodies in their blood. […] Any individual with an associated autoimmune disorder or other condition, especially type 1 diabetes mellitus, autoimmune thyroid disease, autoimmune liver disease, Down syndrome, Turner syndrome, Williams syndrome, and selective immunoglobulin A (IgA) deficiency. […] Total serum IgA: This test is used to check for IgA deficiency, a condition associated with celiac disease that can cause a false negative tTG-IgA or EMA result. If you are IgA deficient, your doctor can order a DGP or tTG-IgG test. […] Deamidated gliadin peptide (DGP IgA and IgG): This test can be used to further screen for celiac disease in individuals with IgA deficiency, which affects 2-3% of patients with celiac disease, or people who test negative for tTg or EMA antibodies. IgA deficiency in a patient may be indicative of other diseases that may cause villus atrophy, such as giardiasis, small-bowel bacterial overgrowth (SIBO) or common variable immunodeficiency (CVID).

• #3 Demographic Features, Clinical, and Laboratory Findings of Partial and Selective IgA Deficiency in Children – The Journal of Pediatric Research

  https://jpedres.org/articles/demographic-features-clinical-and-laboratory-findings-of-partial-and-selective-iga-deficiency-in-children/doi/jpr.galenos.2024.89137

  Following an IgAD diagnosis, patients should undergo close immunological and clinical monitoring. […] Our study observed that some patients diagnosed with partial IgAD could switch to selective IgAD, while others in the selective group could switch to partial deficiency. […] It is important to monitor these transitions and the potential for reversals in the risk assessment and follow-up of these patients. […] This study observed that the patients complaints could begin over a wide age range, and most applied to a physician before 4 years of age. […] The most significant risk factor for the development of IgAD and CVID is a positive family history and it is recommended that the families of IgAD and CVID patients undergo routine screening. […] Our results demonstrate that both selective and partial IgAD patients were mostly struggling with recurrent infections, as previously reported.

• #3 Demographic Features, Clinical, and Laboratory Findings of Partial and Selective IgA Deficiency in Children – The Journal of Pediatric Research

  https://jpedres.org/articles/demographic-features-clinical-and-laboratory-findings-of-partial-and-selective-iga-deficiency-in-children/doi/jpr.galenos.2024.89137

  A substantial number of patients demonstrated reversals in a comprehensive study conducted in Sweden, despite the fact that a diagnosis of IgAD was made after the age of 4 years. […] The association between IgAD and autoimmunity may be explained by the relationship between IgAD and abnormal T-cell regulation, particularly in regulatory T-cells and reduced switching memory B-cells. […] Autoimmunity was present in 14.1% of the participants in our investigation. […] The most prevalent autoimmune condition among our patients was autoimmune thyroiditis, which affected 6.0% of individuals. […] This highlights the high frequency of allergy in IgAD patients and the necessity of vigilant monitoring of these individuals for allergic manifestations during both the diagnosis and follow-up phases. […] Ultimately, patients diagnosed with IgAD should undergo extensive immunological and clinical monitoring during their follow-up.

• #3 Immunoglobulin A Deficiency

  https://www.verywellhealth.com/immunoglobulin-a-deficiency-82726

  Deficiency of immunoglobulin A (IgA) is the most common primary immunodeficiency. It is characterized by very low to absent levels of IgA in the bloodstream. […] For a diagnosis of IgA deficiency, a person must have: Complete absence or extremely low values of IgA measured in the blood; Normal IgG and IgM levels. […] If you have possible celiac disease, you might also have a test to check for IgA deficiency. This can help determine whether a normal test for celiac disease could actually be a false negative result as a consequence of IgA deficiency. […] If you have IgA deficiency, your healthcare provider will monitor you for signs of autoimmune diseases and help guide your management.

• #3 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 prevalence of at least one autoimmunity in SIgAD patients was assessed in 18 studies and ranged from 4.2% by stergaard P.A. from Denmark to 39.2% by Kanoh T., et al. from Japan. The pooled prevalence of autoimmunity in the study populations was 22.0%. Eighteen types of autoimmune manifestations were reported by the current study investigations. Based on meta-analysis results, celiac disease, inflammatory bowel disease (IBD), and rheumatoid arthritis were the most prevalent autoimmunity in SIgAD patients, respectively. […] The prevalence of at least one allergic disease was determined in 16 different studies and fluctuated from 5.50% by Junwu Zhang, et al. from China to 68.25% by Mohammadinejad P, et al., from Iran. The pooled prevalence of at least one allergic disease in the study population was 29.16%. Eight types of allergic diseases were reported by the current study investigations. Based on meta-analysis results, the pooled prevalence of asthma, allergic rhinitis, and allergic conjunctivitis were reported as the most widespread allergic diseases in SIgAD patients.

• #3 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

  Overall, our study identified the infection as the most prevalent clinical presentation among IgAD patients, followed by allergic and autoimmune diseases. Due to the functional role of secretory IgA in maintaining the homeostasis of mucosal surfaces, these complications mainly involved respiratory and GI tracts. 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.

• #4 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). It is classified as a humoral PID characterized by isolated deficiency of IgA (less than 7 mg/dL but normal serum IgG and IgM) in subjects greater than 4 years of age. […] The aim of this review is to examine the association between SIgAD and atopic disease and to update readers on advances over time at this important interface between allergy and SIgAD. […] The criteria for the definitive diagnosis of SIgAD include patients greater than 4 years of age who have serum IgA levels of less than 7 mg/dL (0.07 g/L) but normal serum IgG and IgM (also defined as isolated IgA deficiency), where other causes of hypogammaglobulinemia have been excluded, and there is normal IgG antibody response to vaccination.

• #4 Frontiers | Innate Mechanisms in Selective IgA Deficiency

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

  The diagnostic procedure should include the measurement of serum antibodies of IgM, IgG and IgA class and a complete blood cell count complemented with the measurement of peripheral blood B cells, T cells, and NK cells. […] The diagnosis of SIgAD is therefore often accidental when patients are screened for the presence of other diseases like allergies or autoimmune diseases. […] The SIgAD diagnosis is usually based on the measurements of IgA concentrations in the blood of a patient older than four years being below 0.05 mg/ml (5 mg/dL) while levels of IgG and IgM are normal. […] Therefore, in children aged 6 months to 4 years with low serum IgA levels these measurements should be repeated at 4 years of age to confirm the formal diagnosis of SIgAD. […] Therefore, there is a pressing need to design a diagnostic procedure to rapidly identify these children at younger age and to relieve them of the burden of recurrent infections and the predisposition to develop other associated diseases later in life.

• #4 Selective IgA deficiency – Immunodeficiency UKAccessibilityIncrease TextDecrease TextGrayscaleHigh ContrastNegative ContrastLight BackgroundLinks UnderlineReadable FontReset

  https://www.immunodeficiencyuk.org/selective-iga-deficiency-2/

  Selective immunoglobulin A deficiency (sIgAD) is the most common of the primary antibody deficiencies, affecting up to 1 in 600 people. […] A clinical immunologist usually makes the diagnosis of sIgAD. […] To diagnose sIgAD the specialist immunologist may carry out blood tests to: Measure the levels of the different immunoglobulins: IgA, IgG and IgG subclasses, and IgM. […] If antibodies are not present in the blood, you will be immunised and blood taken three to four weeks later to see if you have responded to the vaccines. […] How is sIgAD diagnosed?

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

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

  Allergy is a quite common clinical complication affecting more than 30% of patients. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. […] The suspicion of SIgAD should heighten not only in patients with recurrent infections but also in those with atopic manifestations. […] Studies analyzing allergy on SIgAD patients showed prevalence ranging from 13 to 84%, supporting in most of the cases the relationship between SIgAD and allergic diseases. […] It is worth mentioning that in many cases allergy is the first symptom of SIgAD, and, in some cases, it is the only symptom of disease. […] The association between SIgAD and allergy was found for various allergic manifestations, the most commonly described of which are asthma, allergic rhinitis, allergic conjunctivitis, urticaria, atopic dermatitis and food allergy, even if the exact prevalence of each symptom and of one clinical manifestation or another varies among the studies reported. […] In light of the association between allergy/asthma and recurrent infections in SIgAD, early detection and management of respiratory disorders is essential to prevent severe complications.

• #4 Immunoglobulin A Deficiency Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Immunoglobulin A deficiency (IgAD) is defined as an undetectable serum IgA level. In the past, this was usually confirmed with the low-level radial immunodiffusion method (lower limit of detection is 50 mg/mL [5 mg/dL]). However, this test is rarely done in current practice, and results are usually reported as 0.07 g/L or 0.05 g/L. The lower limit of detection differs depending on the sensitivity of the method used. It is usually 0.050.1 g/L for nephelometry, 0.05 g/L (5 mg/dL) for low-level radial immunodiffusion plates, and 0.0016 g/L for hemaglutination inhibition techniques. […] […] Normal serum levels of IgG and IgM are necessary for a diagnosis of selective immunoglobulin A deficiency (SIgAD). Other causes of hypogammaglobulinemia and use of medications that reduce IgA levels should be excluded (see Differentials). A total IgG level within the normal range does not exclude specific IgG antibody deficiency (eg, deficient antibodies to polysaccharides), and this should be specifically sought in patients who have recurrent infections, total IgG levels toward the lower bound of normal, and IgA deficiency. Tests showing low IgA serum values in children younger than 5 years should be repeated. Some children with low levels progress to CVID, but levels can normalize by age 4-5 years. […] […] The most common mistake clinicians make with this diagnosis is incorrectly diagnosing IgAD or transient hypogammaglobulinemia of infancy in children because they inappropriately use the adult reference range for serum IgA levels. […]

• #4 Celiac Disease and Iga Deficiency – About a Clinical Case

  https://www.heraldopenaccess.us/openaccess/celiac-disease-and-iga-deficiency-about-a-clinical-case

  The main question and difficulty are knowing how to monitor these patients, that is, how to monitor response to the gluten-free diet (GFD). […] The procedures for diagnosing and monitoring CD in patients with SIgAD still require careful review. […] The only established consensus seems to be the determination of anti-tTG IgA and total serum IgA as an initial screening for all patients with suspected CD. […] Once the presence of SIgAD has been demonstrated, the choice of an IgG-based test necessary to continue the diagnostic investigation remains open. […] There is no strong evidence on which should be the most sensitive and useful IgG marker for both diagnosis and follow-up, given the great heterogeneity of IgG serology in this field. […] Even so, some studies have identified anti-tTG IgG as a more reliable marker for monitoring than anti-DGP IgG for CD in patients with SIgAD, which is the opposite of what we verified with this case.

• #4 Demographic Features, Clinical, and Laboratory Findings of Partial and Selective IgA Deficiency in Children – The Journal of Pediatric Research

  https://jpedres.org/articles/demographic-features-clinical-and-laboratory-findings-of-partial-and-selective-iga-deficiency-in-children/doi/jpr.galenos.2024.89137

  A substantial number of patients demonstrated reversals in a comprehensive study conducted in Sweden, despite the fact that a diagnosis of IgAD was made after the age of 4 years. […] The association between IgAD and autoimmunity may be explained by the relationship between IgAD and abnormal T-cell regulation, particularly in regulatory T-cells and reduced switching memory B-cells. […] Autoimmunity was present in 14.1% of the participants in our investigation. […] The most prevalent autoimmune condition among our patients was autoimmune thyroiditis, which affected 6.0% of individuals. […] This highlights the high frequency of allergy in IgAD patients and the necessity of vigilant monitoring of these individuals for allergic manifestations during both the diagnosis and follow-up phases. […] Ultimately, patients diagnosed with IgAD should undergo extensive immunological and clinical monitoring during their follow-up.

• #4 Immunoglobulin A Deficiency

  https://www.verywellhealth.com/immunoglobulin-a-deficiency-82726

  Deficiency of immunoglobulin A (IgA) is the most common primary immunodeficiency. It is characterized by very low to absent levels of IgA in the bloodstream. […] For a diagnosis of IgA deficiency, a person must have: Complete absence or extremely low values of IgA measured in the blood; Normal IgG and IgM levels. […] If you have possible celiac disease, you might also have a test to check for IgA deficiency. This can help determine whether a normal test for celiac disease could actually be a false negative result as a consequence of IgA deficiency. […] If you have IgA deficiency, your healthcare provider will monitor you for signs of autoimmune diseases and help guide your management.

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