Materiały źródłowe

• #1 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. […] Many IgAD patients are asymptomatic (ie, „normal” blood donors) and are identified by finding a laboratory abnormality, without any apparent associated clinical disease. Some patients with IgAD may have the following associated conditions: (1) deficits in one or more immunoglobulin G (IgG) subclasses (this accounts for 20-30% of IgA-deficient patients, many of whom may have total IgG levels within the normal range) or (2) a deficient antibody response to pneumococcal immunization (specific polysaccharide antibody deficiency [SPAD]). […] Although individuals with IgAD have largely been considered healthy, recent studies indicate a higher rate of symptoms. A 20-year follow-up study that compared 204 healthy blood donors with incidentally identified IgAD to 237 healthy subjects with normal IgA levels demonstrated that 80% of IgAD donors and 50% of control subjects had episodes of infections, drug allergy, or autoimmune or atopic disease. Severe respiratory tract infections occurred in 26% of IgAD subjects, in 24% of subjects with decreased IgA levels, and in 8% of control subjects; however, the incidence of life-threatening infections was not increased.

• #2

  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. Most individuals with IgA deficiency are asymptomatic and identified coincidentally. However, some patients may present with recurrent infections of the respiratory and gastrointestinal tracts, allergic disorders, and autoimmune manifestations. […] There is a wide spectrum of clinical findings in IgA deficiency. Patients with IgA deficiency may be identified among blood bank donors, without any clinical findings. In fact, 85-90% of IgA-deficient individuals are asymptomatic. This high percentage is interesting and still remains a puzzle to be solved since IgA is such a significant immunoglobulin in immune defense. Some patients with IgA deficiency have a tendency to develop recurrent sinopulmonary infections, gastrointestinal infections and disorders, allergies, autoimmune conditions, and malignancies.

• #3 Frontiers | Innate Mechanisms in Selective IgA Deficiency

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

  Selective IgA deficiency (SIgAD), characterized by a serum IgA level below 0.07 mg/ml, while displaying normal serum levels of IgM and IgG antibodies, is the most frequently occurring primary immunodeficiency that reveals itself after the first four years after birth. […] However, recent studies show that newborns and young infants already display clinical manifestations of this condition due to aberrancies in their immune defense. Interestingly, there is a huge heterogeneity in the clinical symptoms of the affected individuals. More than 50% of the affected individuals do not have clinical symptoms, while the individuals that do show clinical symptoms can suffer from mild to severe infections, allergies and autoimmune diseases. […] Even though SIgAD is so prevalent, the underlying cause of SIgAD and its heterogeneity in expression of clinical manifestations is still unknown.

• #4 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. […] Patients who suffer from selective IgA deficiency may have clinical symptoms, but most are largely asymptomatic. […] Recent studies reveal that close to 20-30% of patients with IgA deficiency develop severe respiratory and gastrointestinal tract infections and others are at risk for developing adverse reactions to blood products. […] Patients with IgA deficiency could also suffer from concurrent autoimmune disease. […] The most common manifestations associated with selective IgA deficiency are the recurrent pulmonary infections caused by extracellular encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae.

• #4 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. […] Many IgAD patients are asymptomatic (ie, „normal” blood donors) and are identified by finding a laboratory abnormality, without any apparent associated clinical disease. Some patients with IgAD may have the following associated conditions: (1) deficits in one or more immunoglobulin G (IgG) subclasses (this accounts for 20-30% of IgA-deficient patients, many of whom may have total IgG levels within the normal range) or (2) a deficient antibody response to pneumococcal immunization (specific polysaccharide antibody deficiency [SPAD]). […] Although individuals with IgAD have largely been considered healthy, recent studies indicate a higher rate of symptoms. A 20-year follow-up study that compared 204 healthy blood donors with incidentally identified IgAD to 237 healthy subjects with normal IgA levels demonstrated that 80% of IgAD donors and 50% of control subjects had episodes of infections, drug allergy, or autoimmune or atopic disease. Severe respiratory tract infections occurred in 26% of IgAD subjects, in 24% of subjects with decreased IgA levels, and in 8% of control subjects; however, the incidence of life-threatening infections was not increased.

• #5 Mayo Clinic Health Library – Selective IgA deficiency | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20256724

  Most people with selective IgA deficiency have no symptoms. But some people who have selective IgA deficiency have frequent illness of the airways, lungs and digestive system. […] Some people have illnesses more often than is typical. They also may have a particular illness that returns often. […] People with selective IgA deficiency may have frequent or repeat episodes of the following: Ear infections, particularly in young children. Colds. Sinus infections. Lung illnesses, such as bronchitis or pneumonia. Giardiasis, a parasitic illness of the digestive system that causes diarrhea. […] Children with frequent illnesses may not eat well or may not gain weight typical for their age.

• #6 Selective IgA Deficiency: Symptoms and Diagnosis Info

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

  People with selective IgA deficiency are at a higher risk of infection. The experiences of IgA deficiency can vary greatly. Many adults and children with selective IgA deficiency go undiagnosed because they are never sick enough to see a doctor, while others may develop more severe or recurring problems. […] Some selective IgA deficiency symptoms may include: Repeated ear infections, Sinus infections, Infections of the bronchi (airways of the lungs), Pneumonia, Gastrointestinal infections, Ongoing diarrhea, Allergies and asthma. […] People with selective IgA deficiency may also be more susceptible to autoimmune diseases. About one-third of selective IgA deficiency patients have an autoimmune disease. These diseases cause the immune system to mistakenly attack the body’s healthy tissues and cells. Autoimmune diseases often seen in people with selective IgA deficiency include systemic lupus erythematosus, rheumatoid arthritis, celiac disease, and/or a disorder that causes low platelet counts in the blood.

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

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

  The 20-year longitudinal study of healthy blood donors with incidental findings of IgAD used questionnaires and medical record reviews and found a 3-fold increase in rates of severe childhood respiratory conditions (9% vs 3%), a 4-fold increase in rates of severe adult respiratory conditions (16% vs 4%), a similar increase in recurrent mild respiratory tract infections, and a significant increase in rates of recurrent viral infections (16% vs 1%). […] Patients with IgAD who have a compensatory increase in IgM in their upper respiratory tract secretions and GI fluids tend to be less symptomatic. Note that patients with total IgAD are usually more symptomatic than patients with partial IgAD. […] In children aged 6 months to 4 years, immunoglobulin A deficiency (IgAD) may be transient and resolve permanently by age 5 years; in others, the syndrome may progress to common variable immunodeficiency (CVID). […] Adults with selective immunoglobulin A deficiency (SIgAD) are often asymptomatic; however, up to 90% have frequent bacterial respiratory tract infections.

• #8 Clinical symptoms in adults with selective IgA deficiency: a case-control study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23389234/

  Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency in Caucasians with a prevalence of 1/600 and is generally considered a mild disorder. The SIgAD individuals reported significantly more often contracting various upper and lower respiratory infections, with 8 (25.0 %) having been diagnosed with 1 pneumonia in the preceding two years, compared to one (1.6 %) control (p 0.001). Furthermore, the SIgAD individuals were found to have increased proneness to infections and increased prevalence of allergic diseases and autoimmunity, with a total of 84.4 % being affected by any of these diseases, compared to 47.6 % of the controls (p 0.01). This study challenges the common statement of SIgAD being a mild form of immunodeficiency. […] It also highlights the importance of using matched controls in PID clinical research to better detect clinically important manifestations.

• #9 Selective IgA Deficiency – StatPearls – NCBI Bookshelf

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

  Autoimmune diseases have been observed in roughly 20 to 30% of patients with selective IgA deficiency. […] The prognosis, overall, is typically good. Some patients with IgA deficiency may resolve spontaneously. For others, the repeated bouts of infection and/or inflammation, whether compounded by cancer or autoimmune disease, lead to varying rates of decline in health and a cause for trepidation. […] The most common complications are associated with respiratory infections and malignancies. Following complications are associated with IgA deficiency: Obliterative bronchiolitis; In this entity, pulmonary damage is more pronounced than in bronchiectasis. […] Bronchiectasis; Recurrent lower respiratory infections in IgA deficient patients can result in chronic lung damage, eventually resulting in bronchiectasis.

• #10

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

  Infections of the respiratory system are the most common findings in individuals with IgA deficiency. These infections are mostly due to bacteria, e.g., Haemophilus influenzae and Streptococcus pneumoniae. Some patients may develop end organ damage such as bronchiectasis secondary to recurring or chronic infections. Patients with associated antibody deficiency such as IgG2 subclass deficiency have a higher chance of having more severe infections and complications. […] IgA-deficient individuals have a tendency to develop infections and disorders of the gastrointestinal tract. Giardiasis, malabsorption, lactose intolerance, celiac disease, ulcerative colitis, nodular lymphoid hyperplasia, and malign proliferation are among the associated diseases. […] Allergic disorders appear to be common in patients with IgA deficiency. The reported frequency of allergies varies according to the definitions of both IgA deficiency and allergy and also by the evaluation methods.

• #11 Selective deficiency of IgA | Lima Memorial Health System

  https://www.limamemorial.org/health-library/hie%20multimedia-textonly/1/001476

  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. […] Many people with selective IgA deficiency have no symptoms. If a person does have symptoms, they may include frequent episodes of: Bronchitis (airway infection), Chronic diarrhea, Conjunctivitis (eye infection), Gastrointestinal inflammation, including ulcerative colitis, Crohn disease, and a sprue-like illness, Mouth infection, Otitis media (middle ear infection), Pneumonia (lung infection), Sinusitis (sinus infection), Skin infections, Upper respiratory tract infections. […] Selective IgA deficiency tends to be less severe than many other immunodeficiency diseases. Many people with selective IgA deficiency have no symptoms at all. Some people with IgA deficiency will recover on their own and produce IgA in larger quantities over a period of years.

• #12 Selective IgA Deficiency : Symptoms, Causes and Treatment

  https://www.apollohospitals.com/diseases-and-conditions/selective-iga-deficiency-symptoms-causes-and-treatment/

  Selective IgA deficiency is an immunodeficiency disease. […] About 85 to 90% of IgA-deficient individuals are asymptomatic. Some patients with IgA deficiency tend to develop respiratory infections and diseases such as asthma and pneumonia. […] Gastrointestinal infections and associated disorders, such as giardiasis malabsorption, lactose intolerance, celiac disease, ulcerative colitis, and nodular lymphoid hyperplasia may also develop. […] IgA Deficiency also contributes to allergic disorders. […] Several autoimmune diseases are caused by IgA deficiency, such as chronic inflammatory bowel disease, lupus, and rheumatoid arthritis. […] Patients with IgA Deficiency rarely exhibit symptoms. […] Generally, SIgAD patients do well unless they have significant comorbid diseases. […] Most people with this condition are asymptomatic, and it is hereditary. Deficiency in IgA does not require specific treatment. […] Yes, it is a common symptom of SIgAD.

• #13 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. It is not usually associated with bacterial infections but may be associated with autoimmune or allergic diseases. […] People with IgA deficiency may have allergies (e.g. allergic asthma) or an increased tendency to develop autoimmune conditions, where the immune system attacks itself, and this may result in conditions such as coeliac disease, which affects about 10 per cent of IgA-deficient patients. […] In general, individuals who have sIgAD fall into one of four different types and can present with different symptoms: People who are asymptomatic. This covers the majority of people, in whom a lack of IgA in the blood would not have come to notice had they not had an immunoglobulin test for another reason.

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

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

  It is currently not possible to replace IgA in a person with an IgA deficiency, so treatment focuses on alleviating specific symptoms (such as using antibiotics for infections) and treating the underlying issue itself. […] 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. Therefore when it comes to testing for celiac disease when one is IgA deficient, the following information is important: 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.

• #15

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

  Autoimmune diseases are among the most important clinical manifestations in IgA deficiency. Autoantibodies, such as antibodies against sulfatide, Jo-1, cardiolipin, phosphatidylserine, and collagen can be detected in IgA-deficient patients even if overt clinical disease is not the case. […] The prognosis is good in patients with IgA deficiency if it is not associated with a significant disease. IgA deficiency in children may resolve over time. However, it is also known that IgA deficiency may progress into CVID, which has a less favorable outcome. Therefore, a patient with IgA deficiency, once identified, would deserve a regular follow-up of clinical and immunological findings.

• #16 Selective IgA deficiency | Immune Deficiency Foundation

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

  Individuals with selective IgA deficiency (SIgAD) lack immunoglobulin A (IgA), but they usually have normal amounts of IgG and IgM. […] Those people who are symptomatic may develop a variety of significant clinical problems, including infections, allergies, and autoimmune diseases. […] A common problem in SIgAD is susceptibility to infections. Problems with infections are seen in about half of the individuals with SIgAD. Recurrent ear infections, sinusitis, bronchitis, and pneumonia are the most common infections. Some individuals may develop gastrointestinal infections and other gastrointestinal disorders, such as chronic diarrhea. […] Another problem with SIgAD is the occurrence of autoimmune diseases. These are found in about one-third of individuals who seek medical help. Some of the more frequent autoimmune diseases associated with SIgAD include rheumatoid arthritis, celiac disease, systemic lupus erythematosus, and idiopathic thrombocytopenic purpura (ITP) (low platelet counts).

• #17 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 is also associated with inflammatory bowel disease, including Crohn disease and ulcerative colitis. […] Autoimmune diseases that frequently affect patients with SIGAD include juvenile idiopathic arthritis, systemic lupus erythematosus, dermatomyositis, sarcoidosis, Sjgren syndrome, Evans syndrome, isolated hemolytic anemia, pernicious anemia, isolated idiopathic thrombocytopenic purpura, type 1 diabetes mellitus, Hashimoto thyroiditis, pulmonary hemosiderosis, Addison disease, chronic nephritis, Henoch- Schonlein purpura, and myasthenia gravis. […] Patients with SIGAD are also predisposed to the development of malignancies, including gastric and colonic adenocarcinoma and acute lymphoblastic leukemia. […] Most patients with SIGAD have an uncomplicated clinical course. However, continued long-term follow-up to identify other immunological abnormalities, such as CVID, autoimmune disease, and malignancy, is required.

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

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

  It has been hypothesized that 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. […] Patients with IgA deficiency have been reported to be at higher risk for malignancies, especially gastric and colon adenocarcinoma and lymphoproliferative diseases. However, recent studies do not confirm the data between SIgAD and malignancy, making the association still not conclusive.

• #18 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). Patients with SIgAD can be either asymptomatic or symptomatic with clinical manifestations including allergy, autoimmunity and recurrent infections mainly of the respiratory and gastrointestinal tract. […] It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. Therefore, the suspicion of SIgAD should heighten not only in patients with recurrent infections but also in those with atopic manifestations. […] Patients with SIgAD clinically range from asymptomatic patients, who are diagnosed coincidentally during a laboratory screening, to symptomatic patients with heterogeneous clinical phenotypes and severity of the disease: recurrent infections, mainly of the respiratory and gastrointestinal tract, are the most common finding. Allergy is a quite common clinical complication affecting more than 30% of patients. Finally, autoimmune diseases are associated with SIgAD in up to 20% of subjects.

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

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

  The clinical course of the disease is generally favorable in most patients, being influenced by the clinical manifestation. However, progression to CVID is observed in about 5% of patients. […] The most frequent and relevant clinical manifestations are recurrent infections, especially those affecting the respiratory system and the gastrointestinal tract. […] Recurrent infections of the intestinal tract are also common due to alteration of the protective IgA-mucosal barrier of the gastro-intestinal tract, which facilitates the adhesion of pathogens to the epithelium leading to proliferation and establishment of the parasitosis, mainly Giardia Lamblia. […] A wide variety of autoimmune diseases are also associated with SIgAD, including thrombocytopenic purpura, autoimmune hemolytic anemia, type 1 diabetes mellitus, rheumatoid arthritis, systemic lupus erythematosus, Graves’ disease, celiac disease and vitiligo.

• #19 Selective IgA deficiency | Immune Deficiency Foundation

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

  Allergic disorders are more common among individuals with SIgAD than among the general population. The types of allergies associated with SIgAD include allergic rhinitis, allergic conjunctivitis, eczema, and asthma. Food allergy may also be associated with SIgAD. […] Although SIgAD is usually one of the milder forms of immunodeficiency, it may result in severe disease in a subset of people. Therefore, it is difficult to predict the long-term outcome in an individual with SIgAD, although generally, the prognosis is considered to be very good.

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

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

  Lower IgA may imply your immunity is weakened. […] Having low IgA increases the risk of allergies, infections, and autoimmune diseases. […] 85 to 90% of people with IgA deficiency have no symptoms. Instead, they may only show symptoms from their underlying infections or immune disorders. […] IgA protects against allergies. […] Low IgA levels were found in the lungs of severe asthmatics. Lower IgA levels were associated with more severe symptoms. […] IgA-deficient people more often have allergies, including asthma, allergic rhinitis/conjunctivitis, drug allergy, or food allergy. […] People with IgA deficiency are at a higher risk for infections, including infections of the respiratory system, gut, joints, and urinary tract. […] Those with IgA deficiency have a moderately increased risk of cancer, especially gut cancer. […] Symptoms depend on the underlying cause.

• #21 Frontiers | Innate Mechanisms in Selective IgA Deficiency

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

  The diagnosis of SIgAD is therefore often accidental when patients are screened for the presence of other diseases like allergies or autoimmune diseases. […] The individual variation in SIgAD symptoms can be further explained by the finding of genetic polymorphisms in IL-10 and IL-6 genes. […] In summary, a great heterogeneity exists in the clinical manifestations of individuals with SIgAD. […] The absence of IgA in SIgAD reduces its protective role on mucosal surfaces by preventing the passage of allergens over the epithelium and allowing them to get into contact with the local mucosal immune system. Hence, there is an increased likelihood of sensitization against antigens and for example an increased chance of respiratory tract infections and asthma. […] Overall, the prevalence of cancer in individuals with SIgAD is not very high. […] The large variability in clinical manifestations in SIgAD individuals suggests that there might be multiple and different causal mechanisms involved in the development of SIgAD.

• #21 Frontiers | Innate Mechanisms in Selective IgA Deficiency

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

  Currently, no specific treatment for SIgAD exists and novel therapeutic strategies could be developed based on the discussed information. […] SIgAD is the most frequently described form that is partly genetically based and therefore considered a primary deficiency, although it can also be secondary as it can be induced by exposure to drugs or the result of other diseases. […] The combination of fully developed epithelial barriers, an active innate immune system and the presence of protective maternal antibodies for the first 6 months of age collectively protect the infant against the sudden exposure to microbes. […] SIgA antibodies have the capacity to control intestinal antigens by complexing bacterial antigens for removal or uptake through retrograde transport. […] Individuals that suffer from chronic or recurrent infections, chronic diarrhea, different types of autoimmune diseases or any combination of these and are older than 4 years of age are usually tested for selective IgA deficiency.

• #22 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

  Most people with selective IgA deficiency have few or no symptoms, but some have chronic lung infections, sinusitis, and other disorders. […] Most people with selective IgA deficiency have few or no symptoms. Others develop chronic lung infections, sinusitis, allergies, asthma, nasal polyps, chronic diarrhea, or, rarely, autoimmune disorders, such as systemic lupus erythematosus (lupus) or inflammatory bowel disease (which usually causes gastrointestinal symptoms that often recur or that may be serious). A few people develop common variable immunodeficiency over time. […] Some people improve spontaneously. Life span is usually unaffected. If an autoimmune disorder or another immune disorder (such as common variable immunodeficiency) develops, life span may be shortened.

• #23 Selective IgA deficiency

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

  Patients with selective IgA deficiency may be asymptomatic, mildly ill, or seriously ill (25-50%). Their presentations fall into three broad categories: […] Frequent infections. These can include sinusitis, bronchitis, pneumonia, otitis media, bronchiectasis secondary to multiple respiratory infections, oral infections and conjunctivitis. They may also present with gastrointestinal conditions such as ulcerative colitis, Crohn disease and chronic diarrhoea. […] Autoimmune disease. Autoimmune diseases occur in around 25-33% of patients with selective IgA deficiency. The most frequent are systemic lupus erythematosus, rheumatoid arthritis and thrombocytopenic purpura. […] Allergies and asthma. 10-15% of patients with selective IgA deficiency will also present with a broad range of allergies and/or otherwise unexplained asthma.

• #24 IgA Deficiency – AmeriPharma® Specialty Care

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

  The clinical manifestation of immunoglobulin A deficiency is a predisposition towards recurrent infections, which the body defends by antibody response. More commonly, IgA deficiency may often be asymptomatic (no apparent signs or symptoms). If symptoms are present, they may vary from patient to patient, for example: Some patients experience no illness and appear healthy. Some patients encounter slight to moderate infections. Some patients may become more prone to allergic reactions or asthma from immunoglobulin A deficiency. Some patients encounter frequent infections and may need long-term antibiotic treatment. Some patients may need external IgA therapy in the form of IVIG treatment.

• #25 Selective IGA Deficiency

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

  Most people with selective IgA deficiency do not have any symptoms. […] About 25% to 50% of people with selective IgA deficiency will have complications. Some people with selective IgA deficiency experience repeated infections. […] Most people with selective IgA deficiency are healthy, but some patients experience more frequent or severe infections. […] The prognosis for people with selective IgA deficiency varies by the individual but tends to be good. […] However, some people may develop more severe diseases as a result of the condition than others. Selective IgA deficiency tends to be a lifelong condition, but young children can outgrow the condition within the first few years of life.

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

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

  Most patients with selective IgA deficiency are asymptomatic; others have recurrent sinopulmonary infections, diarrhea, allergies (eg, asthma, associated nasal polyps), or autoimmune disorders (eg, celiac disease, inflammatory bowel disease, systemic lupus erythematosus, chronic active hepatitis). […] A few patients with IgA deficiency develop CVID over time; others improve spontaneously. Prognosis is worse if an autoimmune disorder develops. […] Patients may be asymptomatic or have recurrent infections or autoimmune disorders; some develop CVID over time, but in others, selective IgA deficiency spontaneously resolves.

• #27 FloridaHealthFinder | Selective deficiency of IgA | Health Encyclopedia | FloridaHealthFinder

  https://quality.healthfinder.fl.gov/health-encyclopedia/HIE/1/001476

  Many people with selective IgA deficiency have no symptoms. […] If a person does have symptoms, they may include frequent episodes of: […] Other symptoms include: […] Selective IgA deficiency is less harmful than many other immunodeficiency diseases. […] Some people with IgA deficiency will recover on their own and produce IgA in larger quantities over a period of years.

• #28 Immunodeficiency Search

  https://www.immunodeficiencysearch.com/iga-deficiency

  Selective IgA deficiency is defined as the isolated undetectable serum IgA (with normal IgG and IgM levels) in a patient older than 4 years of age. […] The majority of patients (two-thirds) with selective IgA deficiency are healthy and asymptomatic. These patients do not require any treatment and do not require regular follow up. […] A minority of patients with IgA deficiency are characterized by the following clinical features. Sinopulmonary Infections – Recurrent ear infections, sinus infections, and pneumonias with encapsulated bacteria are the most common infectious complications reported. […] Very rarely, anaphylactic transfusion reactions to plasma-containing blood products may occur. […] In rare cases, patients with selective IgA deficiency may progress to Common Variable Immune Deficiency (CVID). Thus, patients who are symptomatic with sinopulmonary infections should be followed longitudinally to determine if CVID develops. […] Some patients with selective IgA deficiency may progress to Common Variable Immune Deficiency (CVID). Thus, patients who are symptomatic with sinopulmonary infections should be followed longitudinally to determine if CVID develops.

• #29 Evolution of IgA deficiency to IgG subclass deficiency and common variable immunodeficiency | Allergologia et Immunopathologia

  https://www.elsevier.es/en-revista-allergologia-et-immunopathologia-105-articulo-evolution-of-iga-deficiency-to-13011415

  Evolution of IgA deficiency to IgG subclass deficiency and common variable immunodeficiency […] First report: male child with repeated pulmonary infections from the age of 4 months. He was diagnosed as IgA deficiency (undetectable IgA levels) at the age of 3 years, when he presented repeated bouts of pneumonia and tonsillitis. […] At 8 years and 9 months, the diagnosis of IgA deficiency was confirmed, and associated IgG2 and IgG4 deficiency (29.0 mg/dl y 0.01 mg/dl) with normal total IgG serum level was found. […] Second report: a boy with repeated infections since the age of 2 months. IgA deficiency was diagnosed at 1 year 7 months (undetectable serum IgA levels). At age 51/2 years, his clinical course worsened and more serious infections appeared. […] The findings suggest an association of IgA deficiency and common variable immunodeficiency.

• #30 Evolution of IgA deficiency to IgG subclass deficiency and common variable immunodeficiency | Allergologia et Immunopathologia

  https://www.elsevier.es/en-revista-allergologia-et-immunopathologia-105-articulo-evolution-of-iga-deficiency-to-13011415

  Reports of associated deficiencies in IgA and IgG2 and IgG4 subclasses are rare in the literature. The association of IgA and common variable immune deficiency is even less common. […] We conclude that IgA deficiency is not always consistent in terms of the type of immune deficiency present. It is important to follow-up of children with IgA deficiency in order to detect and treat other immune deficiencies that may appear.

• #31 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

  Immunoglobulin A deficiency (IgAD), which is the most common primary antibody deficiency, can cause clinical problems due to significant infections and associated diseases, while some individuals with IgAD remain symptomless throughout their lives. […] The majority of patients experienced infections (56.3%) and allergies (47.7%). […] The most common complaint at admission was frequent infections (n=84, 56.3%), defined as eight or more infection episodes yearly. […] The primary antibody of the mucosal immune system is secretory IgA, and if deficient, microorganisms, aeroallergens, and food-borne allergens easily penetrate the mucosal barrier and enter the body. Therefore, the common complaints are sinopulmonary, gastrointestinal system infections, allergies, and autoimmune disorders. […] Selective and partial IgAD generally have a favorable prognosis, and it is possible for their immunoglobulin levels to rise during the course of their follow-up. However, chronic allergy complaints, autoimmune diseases, and accompanying immunological disorders closely relate to the course of this disease and the severity of infections. […] This study revealed that infections were the most common concern, but the frequencies of allergic manifestations and autoimmunity were also significant. […] Following an IgAD diagnosis, patients should undergo close immunological and clinical monitoring.

• #32 Immunoglobulin A Deficiency | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/i/immunoglobulin-a-deficiency.html

  Most people with an IgA deficiency dont have any symptoms or health problems. […] Some people with an IgA deficiency are more likely to get frequent infections. These problems can include sinus, lung, and digestive infections. […] Some people with IgA deficiency also are more likely to have allergies. They may also have digestive and autoimmune problems, such as celiac disease, rheumatoid arthritis, Graves disease, or lupus. […] Complications for IgA deficiency include asthma, diarrhea, ear and eye infections, autoimmune diseases, and pneumonia.

• #33 Selective IgA Deficiency – StatPearls – NCBI Bookshelf

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

  The most severe complications are related to the association of selective IgA deficiency and the development of malignancies, such as adenocarcinoma of the stomach and lymphoma, which arise in particularly older patients. […] IgA deficiency is felt to be a risk factor for severe COVID-19 infection.

• #34 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. […] However, in rare patients, the deficiency leads to an increased incidence of infection, particularly sinopulmonary infection. […] Up to one-third of patients with SIGAD have recurrent infections (such as recurrent otitis media and sinopulmonary and GI tract infections). […] Symptoms may become apparent in the first year of life. Respiratory tract infections may resolve by adulthood in some patients, whereas in other patients, infections may persist throughout adulthood. […] Rarely, recurrent bronchitis, pneumonia, or even bronchiectasis may occur in patients with SIGAD. […] In patients with SIGAD, the lack of secretory IgA to bind and destroy toxins and intestinal pathogens leads to a defective mucosal barrier. Consequently, children with SIGAD may have GI tract infections, especially from Giardia lamblia.

• #35 Selective Iga Deficiency

  https://www.pediatriconcall.com/articles/immunodeficiencies/selective-iga-deficiency/selective-iga-deficiency-patient-education

  Most patients with selective IgA deficiency have no symptoms. Some people have a similar course as those patients with common variable immunodeficiency (cvid). Some patients may have recurrent Ear Infections, sinusitis, bronchitis, pneumonia, and recurrent diarrhea. About 25% – 33% of patients with selective IgA deficiency may develop autoimmune diseases such as Rheumatoid arthritis, systemic lupus erythematosus (SLE) and immune thrombocytopenic purpura (ITP). Thus, these patients may get arthritis or low blood counts. Allergies are also common in individuals with selective IgA deficiency and asthma, food allergy, anaphylaxis is commonly seen.

• #36 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). […] A common problem in IgA deficiency is susceptibility to infections. […] The most common symptom of Selective IgA Deficiency is susceptibility to infections including: pneumonia, sinusitis, ear infections, chronic diarrhea caused by gastrointestinal infections. […] IgA deficiency may also cause autoimmune disease, in which the immune system attacks itself. Common examples of these diseases are rheumatoid arthritis and lupus. […] In cases where recurrent infections are a problem, preventative antibiotics may be used to help diminish the frequency of infections. Individuals with IgA deficiency often require a longer course of antibiotics for infections to clear up. Selective IgA deficiency can also predispose individuals to have allergic-like or anaphylactic reactions to blood transfusions or infusions of certain blood products.

• #37 What Is Selective IgA Deficiency in Children?

  https://www.icliniq.com/articles/immune-disorders/selective-iga-deficiency-in-children

  IgA deficiency can cause the development of disorders and infections of the gastrointestinal tract. […] Allergic disorders are common in individuals with IgA deficiency. […] Autoimmune diseases are the most important clinical presentations of IgA deficiency. […] IgA-deficient individuals may develop anti-IgA antibodies that can cause anaphylactic reactions with transfusion of any blood derivative, such as red blood cells or platelets that contain trace amounts of IgA. […] The link between IgA deficiency and malignancies is reported in sporadic cases, especially at older ages. These malignancies are usually of gastrointestinal and lymphoid origins. […] While there is no cure for selective IgA deficiency in children, appropriate management strategies help minimize the impact of the condition on their health.

• #38 Selective immunoglobulin A deficiency – Wikipedia

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

  Prognosis is excellent, although there is an association with autoimmune disease. Of note, selective IgA deficiency can complicate the diagnosis of one such condition, celiac disease, as the deficiency masks the high levels of certain IgA antibodies usually seen in celiac disease. As opposed to the related condition CVID, selective IgA deficiency is not associated with an increased risk of cancer. […] Patients with Selective IgA deficiency rarely have severe reactions to blood transfusions. Although Selective IgA deficiency is common, severe reactions to blood transfusions are very rare. People with selective IgA deficiency do not require special blood products unless they have a history of a severe allergic reaction to a blood transfusion.

• #39 IgA deficiency symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/iga-deficiency

  Selective IgA Deficiency is decreased or absent IgA immunoglobulins in the blood serum and secretions. It is one of the most common primary immunodeficiency diseases. […] Common symptom Fatigue […] 17 IgA deficiency patients report severe fatigue (35%) […] 18 IgA deficiency patients report moderate fatigue (37%) […] 10 IgA deficiency patients report mild fatigue (20%) […] 3 IgA deficiency patients report no fatigue (6%). […] Common symptom Pain […] 13 IgA deficiency patients report severe pain (27%) […] 19 IgA deficiency patients report moderate pain (40%) […] 10 IgA deficiency patients report mild pain (21%) […] 5 IgA deficiency patients report no pain (10%). […] Common symptom Stress […] 3 IgA deficiency patients report severe stress (15%) […] 11 IgA deficiency patients report moderate stress (55%)

• #40 IgA deficiency symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/iga-deficiency

  6 IgA deficiency patients report mild stress (30%) […] 0 IgA deficiency patients report no stress (0%). […] Common symptom Anxious mood […] 7 IgA deficiency patients report severe anxious mood (14%) […] 13 IgA deficiency patients report moderate anxious mood (27%) […] 15 IgA deficiency patients report mild anxious mood (31%) […] 13 IgA deficiency patients report no anxious mood (27%). […] Common symptom Depressed mood […] 6 IgA deficiency patients report severe depressed mood (12%) […] 9 IgA deficiency patients report moderate depressed mood (18%) […] 16 IgA deficiency patients report mild depressed mood (33%) […] 17 IgA deficiency patients report no depressed mood (35%).

• #41 IgA Deficiency – AmeriPharma® Specialty Care

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

  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. […] For example, people with selective IgA deficiency may frequently suffer from infections of the respiratory system, gastrointestinal tract, and lungs. This is because IgA is mainly located in the blood and the mucus secretions of these organs and the lack of IgA in these areas increases the risk of infection as there are little to no antibodies to fight off infections. […] Symptoms of IgA Deficiency can include: Fatigue or unusual tiredness (the most common and frequent symptom), Susceptibility to infections, Pneumonia and respiratory infections, Gastrointestinal inflammation, Bacterial infections, Conjunctivitis, Chronic diarrhea, Mouth infection, Bronchitis, Otitis media, Allergies, Asthma.

• #42 Reddit – The heart of the internet

  https://www.reddit.com/r/ChronicIllness/comments/1hlmbun/selective_iga_deficiency_sigad/

  IgA protects your sinuses/airways, your gut, and is transfered to infants during pregnancy/breastfeeding for protection until their own immune systems develop fully. My biggest symptoms are gastroparesis and chronic sinus infections. […] I’ve found very very few personal accounts on living with this immunodeficiency, despite it apparently being the most common one. […] Especially any moms with this, I want to have kids but am afraid that my low immunity will be dangerous for them.

• #43 Low IgA and Selective IgA Deficiency

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

  Selective IgA deficiency (SIgAD) is the most common immunodeficiency worldwide, with an estimated prevalence of 1:400. However, the prevalence varies considerably by ethnicity, being more common in Caucasian populations (about 1%). […] The majority of people with IgA deficiency have no symptoms, but it can increase the risk of intestinal or respiratory infections, as well as allergies. […] Most people with IgA deficiency don’t have any noticeable symptoms, but they may be prone to getting certain types of infections more often. […] Symptoms of IgA deficiency include: Increased gastrointestinal tract infections, Recurrent respiratory tract infections, Increased risk of allergies, More frequent urinary tract infections or skin infections, Increased risk of autoimmune diseases. […] Celiac disease is much more common in people with IgA deficiency. In one study, 6.7% of celiac disease patients had IgA deficiency compared to 0.2% of healthy controls. Type 1 diabetes is also much more common in people with IgA deficiency.

• #44 Primary Antibody Deficiency | Doctor

  https://patient.info/doctor/primary-antibody-deficiency

  Selective IgA deficiency is relatively common in the Caucasian population and causes no symptoms in many of those affected, although some may develop significant clinical problems. It is characterised by undetectable levels of IgA in blood and secretions but no other immunoglobulin deficiencies. […] Patients with primary antibody deficiencies commonly develop recurrent respiratory infections which can lead to bronchiectasis, long-term morbidity and increased mortality. Recognising symptoms and making an early diagnosis is vital to enable timely treatment. […] All clinically significant PADs are characterised by high prevalence of chronic or recurrent infections commonly caused by pyogenic encapsulated bacteria – eg, S. pneumoniae and H. influenzae type b. In addition to common infections, intestinal giardiasis and cryptosporidiosis may be problematic. The possibility of a PAD is suggested by recurrent and severe infections of different types, including sinopulmonary infections (pneumonia, sinusitis, otitis media), meningitis and/or sepsis, gastrointestinal infections (chronic diarrhoea or malabsorption), and cutaneous infections.

• #45 Selective IgA deficiency: Clinical manifestations, pathophysiology, and diagnosis – UpToDate

  https://www.uptodate.com/contents/selective-iga-deficiency-clinical-manifestations-pathophysiology-and-diagnosis

  Selective immunoglobulin A (IgA) deficiency (sIgAD; MIM 137100) may be defined as the isolated deficiency of serum IgA (ie, in the setting of normal serum levels of immunoglobulin G [IgG] and immunoglobulin M [IgM]) in an individual older than four years of age in whom other causes of hypogammaglobulinemia have been excluded. […] The clinical manifestations of sIgAD are variable, ranging from no symptoms to recurrent infections and autoimmune disease.

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