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Clinical manifestations, epidemiology, and diagnosis of common variable immunodeficiency in adults

Sam Ahn, MD
Charlotte Cunningham-Rundles, MD, PhD
Section Editor
E Richard Stiehm, MD
Deputy Editor
Anna M Feldweg, MD


Common variable immunodeficiency (CVID, MIM# 240500) is a primary immunodeficiency disorder characterized by impaired B cell differentiation with defective immunoglobulin production. It is the most prevalent form of severe antibody deficiency affecting both children and adults. "Variable" refers to the heterogeneous clinical manifestations of this disorder, which include recurrent infections, chronic lung disease, autoimmune disorders, gastrointestinal disease, and a heightened susceptibility to lymphoma.

CVID is not a single disease, but rather a collection of hypogammaglobulinemia syndromes resulting from many genetic defects. In a small subset of patients, specific molecular defects have been identified, although in most cases these causes are, as of yet, unknown.

The clinical manifestations, epidemiology, evaluation, and diagnosis of CVID will be discussed here, with a focus on presentation in adult patients. Issues that are particularly relevant to the clinical manifestations and diagnosis in children are reviewed separately. The pathogenesis and treatment in adults are also presented separately. (See "Common variable immunodeficiency in children" and "Pathogenesis of common variable immunodeficiency" and "Treatment and prognosis of common variable immunodeficiency".)


Common variable immunodeficiency (CVID) is defined by the following [1]:

Markedly reduced serum concentrations of immunoglobulin G (IgG), in combination with low levels of immunoglobulin A (IgA) and/or immunoglobulin M (IgM)

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Literature review current through: Nov 2017. | This topic last updated: Apr 25, 2016.
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