title: Inflammatory and autoimmune complications of common variable immune deficiency.
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.
authors.affiliation: Clinical Immunology, Mount Sinai School of Medicine, Room 1120, Box 1089, 1425 Madison Ave, New York 10029, USA. adina.knight@mssm.edu
authors.forename: Adina Kay
authors.initials: AK
authors.lastname: Knight
authors.forename: Charlotte
authors.initials: C
authors.lastname: Cunningham-Rundles
mesh_terms: D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy / Q000209:etiology; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000209:etiology*; D004406:Dysgammaglobulinemia / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006099:Granuloma / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007249:Inflammation / Q000188:drug therapy / Q000209:etiology*; D015212:Inflammatory Bowel Diseases / Q000209:etiology; D011014:Pneumonia / Q000209:etiology; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000209:etiology; D000069283:Rituximab; D012852:Sinusitis / Q000209:etiology
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab; D006886:Hydroxychloroquine