When selective IgA deficiency, the most common primary immunodeficiency, IgA level is <7 mg / dl; IgG and IgM levels are normal. It is the most common primary immunodeficiency. Many patients are asymptomatic, but some develop recurrent infections and autoimmune diseases. Some patients develop a variable immunodeficiency (CVID) with time. Sometimes there can be spontaneous remission. The diagnosis is made by measuring the serum immunoglobulins. The treatment is antibiotics as needed (sometimes prophylactic) and usually contain about avoiding blood products IgA.

(See also Overview of immune deficiency disorders, and approach to the patient with an immunodeficiency disorder.)

When selective IgA deficiency, the most common primary immunodeficiency, IgA level is <7 mg / dl; IgG and IgM levels are normal. It is the most common primary immunodeficiency. Many patients are asymptomatic, but some develop recurrent infections and autoimmune diseases. Some patients develop a variable immunodeficiency (CVID) with time. Sometimes there can be spontaneous remission. The diagnosis is made by measuring the serum immunoglobulins. The treatment is antibiotics as needed (sometimes prophylactic) and usually contain about avoiding blood products IgA. (See also Overview of immune deficiency disorders, and approach to the patient with an immunodeficiency disorder.) IgA deficiency includes B-cell defects. The prevalence ranges from 1/100 to 1 / 1,000. The pattern of inheritance is unknown, but the risk is about 50 times higher in patients who have a family member with selective IgA deficiency. Some patients have mutations in TACI (transmembrane activator and calcium modulator and cyclophilin ligand interactor) gene. A selective IgA deficiency is usually associated with certain HLA haplotypes. Rare alleles or gene deletions in the region desHauptkompatibilitätskomplexes (MHC) are common. Drugs such as phenytoin, sulfasalazine, gold and D-penicillamine can lead to IgA deficiency in some patients. Symptoms and signs Most patients are asymptomatic; some have recurrent infections sinupulmonale, diarrhea, allergies (eg., asthma, nasal polyps associated) or autoimmune diseases (eg. as celiac disease or inflammatory bowel disease, systemic lupus erythematosus, chronic active hepatitis). After contact with IgA in transfusions, immunoglobulin (IVIG) or other blood products may develop anti-IgA antibodies; In rare cases, patients may have anaphylactic reactions when they are exposed to these products again. Diagnosis Clinical evaluation measurement of Ig levels in serum measurement of antibody response to vaccine antigens diagnosis of selective IgA weakness exists in patients with recurrent infections (incl. Giardia infections), anaphylactic transfusion reactions, family history of an ordinary variable immunodeficiency (CVID), IgA deficiency, autoimmune diseases or taking medications that lead to IgA deficiency. The diagnosis is confirmed by a serum IgA levels <7 mg / dl, normal IgG and IgM levels and normal antibody titers to vaccine antigens. A testing of family members is not recommended because most patients have low IgA no clinically significant manifestations. Prognosis Some patients with IgA deficiency develops over time a CVID; in others there is spontaneous remissions. The prognosis is worse if an autoimmune disease develops. Antibiotics treated as needed for the treatment and prophylaxis in severe cases avoidance of blood products that contain IgA are allergic manifestations. Antibiotics are given for bacterial infections of the ears, sinuses, lungs or the gastrointestinal or urogenital tract, as appropriate; in severe cases, they are administered prophylactically. Blood products that contain IgA are avoided, since already been traces can trigger an anti-IgA-mediated anaphylactic reaction. If a red blood cell transfusion is needed only washed compressed erythrocytes may be used. Since an immunoglobulin replacement therapy containing mainly IgG, benefit patients with IgA deficiency is not it; Moreover make anaphylaxis is a risk because the patient may have developed anti-IgA antibodies. In rare cases, specially formulated immunoglobulin preparations which contain very low levels of IgA can be tried and be reasonably effective if the patients have no antibody response to the vaccine and when prophylactic antibiotics are ineffective. Patients are advised to wear an identification bracelet in order to avoid an unintended plasma or immunoglobulin dosing, which could trigger anaphylaxis. Key points The selective IgA deficiency is the most common primary immunodeficiency. Patients may be asymptomatic or have recurrent infections or autoimmune diseases; some patients develop over time CVID, wherein the selective IgA deficiency in other patients but spontaneously disappear. Selective IgA deficiency is suspected when patients have anaphylactic reactions to transfusions, taking medications that lead to IgA deficiency or have recurrent infections or a suggestive family history. The diagnosis is confirmed by measuring the Ig levels and antibody titers after vaccination; an IgA levels <7 mg / dl and normal IgG and IgM levels and antibody titers are diagnostic. Antibiotics are administered prophylactically as needed and in severe cases. The administration of blood products that contain IgA should be avoided in patients.

Health Life Media Team

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