Journal
SEMINARS IN DIAGNOSTIC PATHOLOGY
Volume 29, Issue 4, Pages 177-190Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1053/j.semdp.2012.08.002
Keywords
IgG4-related disease; Autoimmune pancreatitis; Chronic sclerosing sialadenitis; Retroperitoneal fibrosis; Rituximab
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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. (C) 2012 Elsevier Inc. All rights reserved.
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