Inflammatory and autoimmune complications of common variable immune deficiency.
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.
Knight
Adina Kay
AK
Clinical Immunology, Mount Sinai School of Medicine, Room 1120, Box 1089, 1425 Madison Ave, New York 10029, USA. adina.knight@mssm.edu
Cunningham-Rundles
Charlotte
C
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
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab; D006886:Hydroxychloroquine