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Common Variable Immunodeficiency: causes, symptoms, diagnosis, treatment
Medical expert of the article
Last reviewed: 07.07.2025
Common variable immunodeficiency (acquired hypogammaglobulinemia or late-onset hypogammaglobulinemia) is characterized by low Ig levels with phenotypically normal B lymphocytes that are capable of proliferation but do not complete their development by forming Ig-producing cells.
Common variable immunodeficiency (CVID) involves several different molecular defects, but in most patients the molecular defect is unknown. CVID is clinically similar to X-linked agammaglobulinemia in the types of infectious processes that develop, but the onset is later, sometimes in adulthood. In some patients, the T-cell immune response may be impaired. Autoimmune diseases (eg, systemic lupus erythematosus, Addison's disease, thyroiditis, rheumatoid arthritis, alopecia areata, autoimmune hemolytic or pernicious anemia) and malabsorption, nodular lymphoid hyperplasia of the gastrointestinal tract, lymphoid interstitial pneumonia, splenomegaly, and bronchiectasis are common. Gastric carcinoma and lymphoma are found in 10% of patients.
The diagnosis is based on a family history of autoimmune diseases and is confirmed by measuring serum Ig and antibody titers to protein and polysaccharide vaccine antigens. If both are low, flow cytometric B-cell counts are performed to differentiate common variable immunodeficiency from X-linked agammaglobulinemia, multiple myeloma, or chronic lymphocytic leukemia. Serum protein electrophoresis may detect monoclonal gammopathies (eg, myeloma) that are associated with low Ig or other Ig isotypes. Treatment consists of intravenous immunoglobulin 400 mg/kg/month and antibiotic therapy for infections.
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