, Volume 41, Issue 7, pp 626-631,
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Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal

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Introduction

In 1961, Sarles et al.1 asked the following question regarding the particular cases of pancreatitis with hypergammaglobulinemia: “Chronic inflammatory sclerosis of the pancreas—an autoimmune pancreatic disease?” As similar cases were rarely observed, a relationship between such pancreatitis and autoimmunity was viewed skeptically during the following several decades. In 1992, Toki et al.2 have reported 4 cases with unusual diffuse irregular narrowing of the main pancreatic duct and diffuse enlargement of the entire pancreas due to lymphocyte infiltration. In 1995, Japanese investigators3 firstly proposed a concept of “autoimmune pancreatitis (AIP)”, in which the patients showed diffusely enlarged pancreas, narrowing pancreatogram, increased serum IgG, presence of autoantibodies, fibrotic changes with lymphocytic infiltration and steroidal efficacy. Thereafter, many AIP cases have been reported from Japan, and AIP has been accepted as a new clinical entity.4,5 The histopatho

Members of the Autoimmune Pancreatitis Diagnostic Criteria Committee, the Research Committee of Intractable Diseases of the Pancreas, supported by the Japanese Ministry of Health, Labour and Welfare (Chairman, Makoto Otsuki): K. Okazaki, S. Kawa, T. Kamisawa, S. Naruse, S. Tanaka, I. Nishimori, H. Ohara, T. Ito, S. Kiriyama, K. Inui, T. Shimosegawa, M. Koizumi, K. Suda, and M. Otsuki
Members of the Autoimmune Pancreatitis Diagnostic Criteria Committee, the Japan Pancreas Society: K. Shiratori, M. Sugiyama, K. Yamaguchi, and T. Yamaguchi