Immunological aspects of immunodeficiency diseases part 23

Immune 3



Most patients with primary antibody deficiency are collected under the heading  “common variable immune deficiency,” which is condition characterized by low serum IgG and IgA and a variable decrease in IgM and the impaired  production of specific antibodies following natural microbial exposure  or immunization. The estimated prevalence of CVID is between 1 in 10,000 and 1 in 50,000. Clinically, CVID is dominated by the effects of antibody deficiency. About 20 percent of patients with CVID develop autoimmune disorders (arthritis, cytopenias, endocrinopathies). Some patients with CVID develop noncaseating sarcoidlike granulomata infiltrating various organs (lungs, liver, spleen, skin). The mechanisms underlying autoimmunity and granulomatous  disease in CVID are unknown.

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