title: Autoimmune hepatitis in a demographically isolated area of Australia.
abstract: BACKGROUND
  Previous studies describing autoimmune hepatitis (AIH) come from liver transplant centres in which a skewed distribution of cases may give a misleading picture of the incidence of AIH and its natural history. This series describes AIH in a stable and demographically discrete population of patients in the Australian Capital Territory (ACT) and the surrounding region.
  
  
  METHODS
  In 42 patients with type 1 AIH (point prevalence 8 per 100,000 population), clinical, laboratory and histological features at presentation, response to initial therapy, details of maintenance therapy and outcome were recorded.
  
  
  RESULTS
  Consistent with other publications, the male-to-female ratio was 1:3, mean age at presentation was 53 years and 24% had cirrhosis at diagnosis. Most patients (86%) responded to initial therapy and 67% went into long-term remission. One patient died from liver failure and none required liver transplantation. Azathioprine was included in the treatment regimen in 74% of cases with doses generally <2 mg/kg. Azathioprine dose greater than or equal to 2 mg/kg was associated with better clinical outcome, but this did not reach statistical significance. A higher proportion of female patients had cirrhosis at presentation (9/10 vs 1/10; P= 0.24).
  
  
  CONCLUSIONS
  In this Australian community-based study, type 1 AIH was primarily a disease of later life, responded to conventional immunosuppressive therapy and generally has a good prognosis. Further study of the use of azathioprine is warranted to determine the optimal dose.
authors.affiliation: Gastroenterology and Hepatology Unit, The Canberra Hospital, Australian National University, Canberra ACT, Australia.
authors.forename: A S
authors.initials: AS
authors.lastname: Haider
authors.forename: G
authors.initials: G
authors.lastname: Kaye
authors.forename: A
authors.initials: A
authors.lastname: Thomson
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001315:Australia / Q000453:epidemiology; D020958:Australian Capital Territory / Q000453:epidemiology; D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D006801:Humans; D008103:Liver Cirrhosis / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012111:Residence Characteristics; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D001379:Azathioprine