Takahiro Nakazawa1, Kenji Notohara2, Susumu Tazuma3, Atsushi Tanaka4, Hiroyuki Isayama5, Toshio Tsuyuguchi6, Toshiyuki Mori7, Hajime Takikawa4. 1. Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, 2-9 Myoken-cho Showa-ku, Nagoya, 466-8650, Japan. tnakazaw@nagoya2.jrc.or.jp. 2. Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan. 3. Department of General Internal Medicine, Hiroshima University Hospital and Graduate School of Biomedical and Health Science, Hiroshima, Japan. 4. Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan. 5. Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. 6. Department of Gastroenterology and Nephrology, Graduate School of Medicine, Chiba University, Chiba, Japan. 7. Department of Surgery, Kyorin University School of Medicine, Tokyo, Japan.
Abstract
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause that is characterized pathologically by an inflammatory and fibrotic process centered on the epithelium, leading to diffuse biliary stenosis and increased wall thickness throughout the intra- and extra-hepatic biliary trees. A nationwide survey in Japan revealed several differences in the clinical aspects of PSC between Japan and Western countries. PSC was recently designated an intractable hepatobiliary disease in Japan. The aim of this study was to establish Japanese diagnostic criteria for PSC according to the current Japanese conditions. METHODS: The Intractable Hepato-Biliary Diseases Study Group in Japan of the Committee of Research on Measures for Intractable Diseases established a working group consisting of researchers specializing in PSC and proposed the 2016 Japanese diagnostic criteria for PSC. RESULTS: The diagnostic criteria consisted of the major and minor items. Major items are directly related to bile duct changes based on biliary tract imaging and an increased alkaline phosphatase level. Minor items consist of an association with inflammatory bowel disease and liver histology. CONCLUSION: The diagnostic criteria for PSC are useful for general physicians in practice.
BACKGROUND:Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause that is characterized pathologically by an inflammatory and fibrotic process centered on the epithelium, leading to diffuse biliary stenosis and increased wall thickness throughout the intra- and extra-hepatic biliary trees. A nationwide survey in Japan revealed several differences in the clinical aspects of PSC between Japan and Western countries. PSC was recently designated an intractable hepatobiliary disease in Japan. The aim of this study was to establish Japanese diagnostic criteria for PSC according to the current Japanese conditions. METHODS: The Intractable Hepato-Biliary Diseases Study Group in Japan of the Committee of Research on Measures for Intractable Diseases established a working group consisting of researchers specializing in PSC and proposed the 2016 Japanese diagnostic criteria for PSC. RESULTS: The diagnostic criteria consisted of the major and minor items. Major items are directly related to bile duct changes based on biliary tract imaging and an increased alkaline phosphatase level. Minor items consist of an association with inflammatory bowel disease and liver histology. CONCLUSION: The diagnostic criteria for PSC are useful for general physicians in practice.
Authors: Kirsten Boonstra; Karel J van Erpecum; Karin M J van Nieuwkerk; Joost P H Drenth; Alexander C Poen; Ben J M Witteman; Hans A R E Tuynman; Ulrich Beuers; Cyriel Y Ponsioen Journal: Inflamm Bowel Dis Date: 2012-03-08 Impact factor: 5.325
Authors: Einar Björnsson; Rolf Olsson; Annika Bergquist; Stefan Lindgren; Barbara Braden; Roger W Chapman; Kirsten M Boberg; Paul Angulo Journal: Gastroenterology Date: 2008-01-17 Impact factor: 22.682