title: Clinical features of cirrhosis in Japanese patients with type I autoimmune hepatitis.
abstract: OBJECTIVE
  Autoimmune hepatitis (AIH) is a chronic inflammatory disorder of unknown etiology that may proceed to cirrhosis, although some patients already have cirrhosis at the time of AIH diagnosis. The aim of this study was to clarify the clinical characteristics of AIH patients with cirrhosis in Japan.
  
  
  METHODS
  Questionnaires were sent to liver specialists at four research facilities. Data for 250 patients diagnosed with AIH using the scoring system of the International Autoimmune Hepatitis Group (IAIHG) between 1975 and 2010 were collected and analyzed.
  
  
  RESULTS
  The male-to-female ratio was 1:8.3 and the average patient age was 55.6 years. Liver cirrhosis was found in 51 AIH patients (20.4%). Of these, 43 patients (84.3%) had cirrhosis at presentation and eight patients (15.7%) developed cirrhosis during the follow-up period (average follow-up of 82.1 months). There were significant differences between the two groups with and without cirrhosis at presentation with regard to age and biochemical parameters at presentation. There were no significant differences in histology, with the exception of liver fibrosis. The overall 10-year probability of survival was 71.2% vs. 99.3% in the patients with and without cirrhosis (log-rank test, p<0.001). The relapse rate was significantly higher in the patients who developed cirrhosis during treatment than in those who did not develop cirrhosis during treatment (100% vs. 7.5%, p<0.001).
  
  
  CONCLUSIONS
  Since liver cirrhosis has already developed at presentation in many AIH patients with cirrhosis, it is important to diagnose the disease in the early stage and administer treatment rapidly with corticosteroids or immunosuppressants. In addition, a history of relapse is a risk factor for the development of cirrhosis in Japanese patients with AIH.
authors.affiliation: Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, Japan. k-abe@fmu.ac.jp
authors.forename: Kazumichi
authors.initials: K
authors.lastname: Abe
authors.forename: Fumiko
authors.initials: F
authors.lastname: Katsushima
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Kanno
authors.forename: Atsushi
authors.initials: A
authors.lastname: Takahashi
authors.forename: Junko
authors.initials: J
authors.lastname: Yokokawa
authors.forename: Hiromasa
authors.initials: H
authors.lastname: Ohira
authors.forename: Teruko
authors.initials: T
authors.lastname: Arinaga
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Ide
authors.forename: Junko
authors.initials: J
authors.lastname: Nishimura
authors.forename: Megumi
authors.initials: M
authors.lastname: Inoue
authors.forename: Masataka
authors.initials: M
authors.lastname: Seike
authors.forename: Fumio
authors.initials: F
authors.lastname: Imazeki
authors.forename: Osamu
authors.initials: O
authors.lastname: Yokosuka
authors.forename: Michio
authors.initials: M
authors.lastname: Sata
mesh_terms: D044466:Asian People; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications*; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications* / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies