Summary
Selective deficiency of immunoglobulin A (IgAD) is a primary (or congenital) immunodeficiency disease and is the most frequent primary antibody deficiency. With an incidence of approximately 1/325 individuals in western countries, it is not a rare disease. Individuals with IgAD have a partial or total absence IgA, key players in mucosal protection, and thus present with recurrent respiratory tract and gastrointestinal infections. They can also present with common variable immunodeficiency (CVID). An increased incidence of autoimmune disorders is observed in IgAD patients. Aetiology is unknown, but familial inheritance of IgAD has been described in approximately 20-25% of cases and suggests a genetic influence. There is no specific cure for IgAD but efficient treatment of infections is important. Patients with total IgAD should warn their physician in case of blood transfusion, as there is a risk of anaphylactic reaction against IgA-containing blood products. *Author: Orphanet (May 2006)*.
