title: The 2016 diagnostic criteria for primary sclerosing cholangitis.
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.
  
  
  CONCLUSIONS
  The diagnostic criteria for PSC are useful for general physicians in practice.
authors.affiliation: Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, 2-9 Myoken-cho Showa-ku, Nagoya, 466-8650, Japan. tnakazaw@nagoya2.jrc.or.jp.
authors.forename: Takahiro
authors.identifier: http://orcid.org/0000-0002-6321-5995
authors.initials: T
authors.lastname: Nakazawa
authors.affiliation: Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Notohara
authors.affiliation: Department of General Internal Medicine, Hiroshima University Hospital and Graduate School of Biomedical and Health Science, Hiroshima, Japan.
authors.forename: Susumu
authors.initials: S
authors.lastname: Tazuma
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Tanaka
authors.affiliation: Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Isayama
authors.affiliation: Department of Gastroenterology and Nephrology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Toshio
authors.initials: T
authors.lastname: Tsuyuguchi
authors.affiliation: Department of Surgery, Kyorin University School of Medicine, Tokyo, Japan.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Mori
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Takikawa
mesh_terms: D000469:Alkaline Phosphatase / Q000097:blood; D002760:Cholangiopancreatography, Endoscopic Retrograde; D049448:Cholangiopancreatography, Magnetic Resonance; D015209:Cholangitis, Sclerosing / Q000097:blood* / Q000150:complications / Q000000981:diagnostic imaging*; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000150:complications; D007564:Japan; D008099:Liver / Q000473:pathology
chemical_list: D000469:Alkaline Phosphatase
keywords: PSC; Primary sclerosing cholangitis; Sclerosing cholangitis