IgG4-related disease: nomenclature, clinical features, and treatment.
Concepts about IgG4-related disease (IgG4-RD) are now emerging swiftly. The condition has been identified in virtually every organ system, and its features are often excellent mimickers of malignancies, infections, and other immune-mediated disorders. Recommendations for nomenclature were proposed by the Organizing Committee of the 2011 International IgG4-related disease Symposium, and guidelines for the pathologic diagnosis of this condition have been published by an international group of experts. Experience with treatment regimens is growing. Glucocorticoids and B-cell depletion strategies both appear to be effective and are the subject of ongoing studies. This article reviews the current thought and understanding of this disease with regard to nomenclature, organ system involvement, and approaches to therapy.
Rheumatology Unit, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts 02114, USA. jhstone@partners.org
John H
JH
Stone
D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology / Q000378:metabolism / Q000628:therapy; D001402:B-Lymphocytes / Q000187:drug effects / Q000378:metabolism / Q000473:pathology; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008212:Lymphocyte Depletion / Q000379:methods; D008297:Male; D050500:Pancreatitis, Chronic / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D017410:Practice Guidelines as Topic; D012185:Retroperitoneal Fibrosis / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D000069283:Rituximab; D012598:Sclerosis; D012793:Sialadenitis / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D009626:Terminology as Topic
D058846:Antibodies, Monoclonal, Murine-Derived; D005938:Glucocorticoids; D007074:Immunoglobulin G; D000069283:Rituximab