title: Clinical profile of primary biliary cirrhosis that was diagnosed as symptomatic primary biliary cirrhosis according to the revised diagnostic criteria in Japan.
abstract: OBJECTIVE
  The diagnostic criteria for primary biliary cirrhosis (PBC) in Japan were revised in 2004. The prevalence and prognosis of PBC using the revised criteria have not been reported. This study investigated the prevalence and prognosis of "newly-diagnosed" symptomatic-PBC (ns-PBC), which was defined as asymptomatic PBC (a-PBC) in Japan until 2004.
  
  
  METHODS
  The clinical features and the prognosis of 207 patients with PBC were retrospectively investigated according to clinical stage.
  
  
  RESULTS
  The prevalence of ns-PBC was 3.4% and 9.7%, at the time of diagnosis and final evaluation, respectively. The prognosis of ns-PBC was poorer than a-PBC. A total of 7.2% of the patients with a-PBC progressed to ns-PBC during the observation period. These patients had a poorer prognosis than patients who remained asymptomatic.
  
  
  CONCLUSIONS
  Approximately 10% of the PBC patients presented with signs of portal hypertension as an initial symptom. The signs of portal hypertension should therefore be carefully investigated in patients with PBC at the time of diagnosis and during the observation.
authors.affiliation: Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime.
authors.forename: Nobuaki
authors.initials: N
authors.lastname: Azemoto
authors.forename: Masanori
authors.initials: M
authors.lastname: Abe
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Murata
authors.forename: Hidehiro
authors.initials: H
authors.lastname: Murakami
authors.forename: Bunzo
authors.initials: B
authors.lastname: Matsuura
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Hiasa
authors.forename: Morikazu
authors.initials: M
authors.lastname: Onji
mesh_terms: D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis* / Q000453:epidemiology*; D007564:Japan / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis* / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies