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Geoepidemiology of primary sclerosing cholangitis: A critical review
Affiliation:1. Department of Public Health Sciences, Henry Ford Health System, Detroit, Michigan;123. Center for Health Policy and Health Services Research, Henry Ford Health System, Detroit, Michigan;84. Department of Gastroenterology and Hepatology, Henry Ford Health System, Detroit, Michigan;3. Essentia Institute of Rural Health, Essentia Health, Duluth, Minnesota;4. Palo Alto Medical Foundation Research Institute, Palo Alto, California;6. Marshfield Clinic Research Foundation, Marshfield, Wisconsin;5. Center for Health Research, Kaiser Permanente Mid-Atlantic Research Institute, Rockville, Maryland;7. Department of Epidemiology and Health Services Research, Geisinger Clinic, Danville, Pennsylvania;12. Center for Health Research, Kaiser Permanente Northwest, Portland, Oregon;8. Center for Health Research Hawai’i, Kaiser Permanente, Honolulu, Hawaii;10. Department of Research and Evaluation, Kaiser Permanente Southern California, Los Angeles, California;9. Baylor, Scott and White Research Institute, Temple, Texas;71. College of Health Solutions, Arizona State University, Phoenix, Arizona;1. Department of Movement, Human and Health Sciences, Division of Health Sciences, University of Rome “Foro Italico”, Rome, Italy;2. Department of Medico-Surgical Sciences and Biotechnologies, Polo Pontino, Italy;3. Department of Anatomical, Histological, Forensic Medicine and Orthopedics Sciences, Italy;4. Department of General Surgery and Organ Transplantation, Sapienza University of Rome, Rome, Italy;5. Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Cancer, Surgery and Transplantation, Oslo University Hospital Rikshospitalet, Norway;6. Research Institute of Internal Medicine, Division of Cancer, Surgery and Transplantation, Oslo University Hospital Rikshospitalet, Norway;7. K.G. Jebsen Inflammation Research Centre, Institute of Clinical Medicine, University of Oslo, Oslo, Norway;3. European Reference Network ERN RARE-LIVER;1. Fellow Programa Enfermedad Inflamatoria Universidad de Chile-Clínica Las Condes. Servicio de Gastroenterología, Hospital San Juan de Dios, Santiago, Chile;2. Programa Enfermedad Inflamatoria;3. Servicio de Gastroenterología, Clínica Las Condes, Santiago, Chile;4. Servicio de Aparato Digestivo, Hospital Clínico Universitario Lozano Blesa, IIS Aragón, Ciberehd, Zaragoza, España
Abstract:Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin, characterized by progressive destruction of bile ducts caused by diffuse inflammation and fibrosis. Previous epidemiological studies in Northern Europe and North America demonstrated that incidence and prevalence rates are ranging from 0.5 to 1.3 and from 3.85 to 16.2 per 100,000 inhabitants per year, respectively. It is of note that the incidence of PSC appears to be gradually increasing. We have extensively reviewed the geoepidemiology of PSC and attempted to place it in context with the incidence in Japan. In 2012, the clinical diagnostic criteria of IgG4-SC were established and published by the Japan Biliary Association, rendering it possible for physicians to clinically differentiate PSC from IgG4-SC. We conducted a new nationwide survey for PSC as well as IgG4-SC, and have identified 197 patients with PSC and 43 patients with IgG4-SC without pancreatic involvement. In this survey we estimated prevalence rate of PSC in Japan as 0.95, lower than those in North America and European countries. Also we identified other unique features of Japanese PSC patients, including 2 peaks in age distribution at diagnosis and fewer presences of comorbid inflammatory bowel diseases, occurring in only 34% of PSC. This data is placed in the perspective of the international experience on PSC.
Keywords:Epidemiology  Incidence  Prevalence  IgG4-sclerosing cholangitis  PSC"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0035"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  primary sclerosing cholangitis  AIP"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0045"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  autoimmune pancreatitis  IgG4-SCI"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0055"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  gG4-related sclerosing cholangitis  IBD"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0065"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  inflammatory bowel diseases  CCA"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0075"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  cholangiocarcinoma  UC"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0085"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  ulcerative colitis
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