Abstract
Common variable immune deficiency (CVID) is associated with autoimmune and inflammatory complications in addition to recurrent infections. The most common conditions are idiopathic thrombocytopenia purpura, autoimmune hemolytic anemia, sarcoid-like granulomatous disease and gastrointestinal inflammation. IVIG administration reduces the frequency of infections, but does not always prevent autoimmunity or inflammation. TNF antagonists and anti-CD20 immunomodulators have shown some efficacy in CVID in a few patients; further controlled studies are needed to determine the best management of these conditions in the setting of immunodeficiency.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Adult
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Anemia, Hemolytic, Autoimmune / drug therapy
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Anemia, Hemolytic, Autoimmune / etiology
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Antibodies, Monoclonal / therapeutic use
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Antibodies, Monoclonal, Murine-Derived
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Autoimmune Diseases / drug therapy
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Autoimmune Diseases / etiology*
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Dysgammaglobulinemia / complications*
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Dysgammaglobulinemia / drug therapy
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Female
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Granuloma / drug therapy
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Granuloma / etiology
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Humans
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Hydroxychloroquine / therapeutic use
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Immunoglobulins, Intravenous / therapeutic use
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Immunosuppressive Agents / therapeutic use
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Inflammation / drug therapy
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Inflammation / etiology*
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Inflammatory Bowel Diseases / etiology
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Pneumonia / etiology
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Purpura, Thrombocytopenic, Idiopathic / etiology
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Rituximab
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Sinusitis / etiology
Substances
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Antibodies, Monoclonal
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Antibodies, Monoclonal, Murine-Derived
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Immunoglobulins, Intravenous
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Immunosuppressive Agents
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Rituximab
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Hydroxychloroquine