Editorial
Copyright ©2008 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. Aug 28, 2008; 14(32): 4992-4994
Published online Aug 28, 2008. doi: 10.3748/wjg.14.4992
Diagnostic criteria for autoimmune pancreatitis in Japan
Terumi Kamisawa, Kazuichi Okazaki, Shigeyuki Kawa
Terumi Kamisawa, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo 113-8677, Japan
Kazuichi Okazaki, Third Department of Internal Medicine, Kansai Medical University, Osaka 573-1191, Japan
Shigeyuki Kawa, Center for Health, Safety and Environmental Management, Shinshu University, Matsumoto, 390-8621 Japan
Author contributions: Kamisawa T, Okazaki K and Kawa S contributed equally to this work; Kamisawa T wrote the paper.
Supported by Research for Intractable Disease of the Pancreas, Ministry of Health, Labor and Welfare of Japan
Correspondence to: Terumi Kamisawa, MD, PhD, Depart-ment of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan. kamisawa@cick.jp
Telephone: +81-3-38232101 Fax: +81-3-38241552
Received: March 31, 2008
Revised: May 7, 2008
Accepted: May 14, 2008
Published online: August 28, 2008
Abstract

Autoimmune pancreatitis (AIP) is a particular type of pancreatitis of presumed autoimmune etiology. Currently, AIP should be diagnosed based on combination of clinical, serological, morphological, and histopathological features. When diagnosing AIP, it is most important to differentiate it from pancreatic cancer. Diagnostic criteria for AIP, proposed by the Japan Pancreas Society in 2002 first in the world, were revised in 2006. The criteria are based on the minimum consensus of AIP and aim to avoid misdiagnosing pancreatic cancer as far as possible, but not for screening AIP. The criteria consist of the following radiological, serological, and histopathological items: (1) radiological imaging showing narrowing of the main pancreatic duct and enlargement of the pancreas, which are characteristic of the disease; (2) laboratory data showing abnormally elevated levels of serum γ-globulin, IgG or IgG4, or the presence of autoantibodies; (3) histopathological examination of the pancreas demonstrating marked fibrosis and prominent infiltration of lymphocytes and plasma cells, which is called lymphoplasmacytic sclerosing pancreatitis (LPSP). For a diagnosis of AIP, criterion 1 must be present, together with criterion 2 and/or criterion 3. However, it is necessary to exclude malignant diseases such as pancreatic or biliary cancer.

Keywords: Autoimmune pancreatitis, Diagnostic criteria, IgG4, Lymphoplasmacytic sclerosing pancreatitis