Because a diagnostic serological marker is unavailable, autoimmune pancreatitis (AIP) is diagnosed based on unique features. The diagnostic capabilities and potential limitations of four sets of diagnostic criteria for AIP (Japanese diagnostic criteria 2006 and 2011, Asian diagnostic criteria, and international consensus diagnostic criteria (ICDC)) were compared among 85 patients who were diagnosed AIP according to at least one of the four sets. AIP was diagnosed in 87%, 95%, 95%, and 95% of the patients according to the Japanese 2006, Asian, ICDC, and Japanese 2011 criteria, respectively. The ICDC can diagnose types 1 and 2 AIP independently and show high sensitivity for diagnosis of AIP. However, as the ICDC are rather complex, diagnostic criteria for AIP should perhaps be revised and tailored to each country based on the ICDC. 1. Introduction Autoimmune pancreatitis (AIP) is a specific type of pancreatitis that is thought to have an autoimmune etiology. Since Yoshida et al. [1] proposed AIP as a diagnostic entity in 1995, AIP in various countries, including Japan, has been described. AIP is presently recognized as a pancreatic lesion of IgG4-related disease [2, 3]. Because a diagnostic serological marker is unavailable, it is diagnosed based on unique features. The Japanese diagnostic criteria for AIP were revised in 2006 [4]. The criteria consisted of the following: radiological evidence of pancreatic enlargement and irregular narrowing of the main pancreatic duct; increased serum levels of gammaglobulin, IgG, and IgG4 or the presence of autoantibodies; histological evidence of both lymphoplasmacytic infiltration and fibrosis in the pancreas (Table 1) [4]. Table 1: Japanese clinical diagnostic criteria of AIP 2006 [ 4]. New diagnostic criteria proposed in Korea [5] and the USA [6] during 2006 included response to steroid therapy and other organ involvement (OOI). The Asian diagnostic criteria that included response to steroids as an optional criterion were published in 2008 (Table 2) [7]. Table 2: Asian diagnostic criteria for AIP [ 7]. AIP comprises histological types 1 and 2 [8, 9]. The histological appearance of type 1 AIP, or traditional AIP, is referred to as lymphoplasmacytic sclerosing pancreatitis (LPSP). Type 2 AIP is histologically characterized by neutrophilic infiltration in the epithelium of the pancreatic duct [8–10]. The international consensus diagnostic criteria (ICDC) for AIP were published for worldwide use and independently diagnose both types of AIP [11]. The criteria comprise five cardinal features, and one or more of which in
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