Printable factfile
Many individuals with IgA deficiency are asymptomatic. Patients with IgA deficiency have an increased incidence of upper respiratory tract infections, allergies and autoimmune disease like SLE, rheumatoid arthritis, and juvenile chronic arthritis. Others have persistent or recurrent infections and some develop CVI over time. Also, there is a possibility of transfusion reaction due to anti-IgA antibodies. Malignancy may be increased.
Diagnosis
Diagnostic recommendations
Additional Information
Therapeutic options
- Treatment is directed at the presenting disease. Antibiotic therapy (10-14 days) together with physiotherapy and postural drainage in case of lung damage. Intravenous immunoglobulins (200-600 mg/kg/month at intervals of 2-3 weeks) with a low IgA content in case it is needed. It should be avoid IgA-containing products. Associated autoimmune diseases should be treated conventionally.
- Immunoglobulin A deficiency, eMedicine
- Hypogammaglobulinemia, eMedicine
- IgA and IgG subclass deficiencies, eMedicine
Research programs, clinical trials
- European Initiative for Primary Immunodeficiencies
- Improved Healthcare for Patients with Primary Antibody Deficiencies through new Strategies Elucidating their Pathophysiology (IMPAD), IMPAD
- The Genetics of IgA Deficiency and Common Variable Immune Deficiency, Comprehensive Cancer Center
- Immune Regulation in Patients with Common Variable Immunodeficiency and Related Syndromes, ClinicalTrials.gov