Systemic disorders with rheumatic manifestations: Edited by John Stone and Arezou KhosroshahiAutoimmune pancreatitis and IgG4-related sclerosing cholangitisTakuma, Kensukea; Kamisawa, Terumia; Igarashi, YoshinoribAuthor Information aDepartment of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Japan bDepartment of Gastroenterology and Hepatology, Omori Medical Center, Toho University School of Medicine, Tokyo, Japan Correspondence to Terumi Kamisawa, MD, PhD, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan Tel: +81 3 3823 2101; fax: +81 3 3824 1552; e-mail: email@example.com Current Opinion in Rheumatology: January 2011 - Volume 23 - Issue 1 - p 80-87 doi: 10.1097/BOR.0b013e3283412f60 Buy Metrics Abstract Purpose of review Autoimmune pancreatitis (AIP) is a peculiar type of pancreatitis with a presumed autoimmune etiology. AIP is frequently associated with stenosis of the bile duct in the form of IgG4-related sclerosing cholangitis. This article reviews recent advances in clinicopathological findings for AIP and IgG4-related sclerosing cholangitis. Recent findings AIP is currently diagnosed based on characteristic radiological findings (irregular narrowing of the main pancreatic duct and enlargement of the pancreas) in combination with serological findings (elevated serum IgG4 and presence of autoantibodies) and histopathological findings (dense infiltration of IgG4-positive plasma cells and lymphocytes with fibrosis and obliterative phlebitis in the pancreas). Other clinical characteristics include preponderance toward elderly men, common initial symptoms of obstructive jaundice, and favorable response to steroid therapy. Differentiation of AIP from pancreatic cancer is crucial. As AIP is frequently associated with various sclerosing extrapancreatic lesions showing the same peculiar histological findings seen in the pancreas, AIP is currently considered to represent a pancreatic manifestation of IgG4-related sclerosing disease. Considering the age of onset, associated diseases, cholangiography, serum IgG4 levels, and steroid responsiveness, IgG4-related sclerosing cholangitis differs from primary sclerosing cholangitis. Summary AIP and associated extrapancreatic lesions are considered to represent clinical manifestations of IgG4-related sclerosing disease. © 2011 Lippincott Williams & Wilkins, Inc.