title: Characteristics and long-term outcome of patients with autoimmune hepatitis related to the initial treatment response.
abstract: OBJECTIVE
  Autoimmune hepatitis (AIH) is a liver disease which, if untreated, may lead to liver cirrhosis and hepatic failure. Limited data exist regarding factors predicting the long-term outcome. The aims of this study were to investigate symptoms at presentation, prognostic features, management and treatment in relation to long-term outcome of AIH.
  
  
  METHODS
  A cohort of 473 Swedish patients with AIH was characterized regarding initial symptoms and signs, factors predicting death and future need for liver transplantation. Survival and causes of death were retrieved from Swedish national registers.
  
  
  RESULTS
  At diagnosis, fatigue was a predominant symptom (69%), 47% of the patients were jaundiced and 30% had liver cirrhosis. Another 10% developed cirrhosis during follow-up. Markedly elevated alanine aminotransferase levels at presentation were correlated with a better outcome. A high international normalized ratio (INR) at diagnosis was the only risk factor predicting a need for later liver transplantation. Histological cirrhosis, decompensation and non-response to initial treatment were all factors that correlated with a worse outcome. Overall life expectancy was generally favourable. However, most deaths were liver-related, e.g. liver failure, shock and gastrointestinal bleeding.
  
  
  CONCLUSIONS
  Cirrhosis at diagnosis, a non-response to initial immune-suppressive treatment or elevated INR values were associated with worse outcome and a need for later liver transplantation. In contrast, an acute hepatitis-like onset with intact synthetic capacity indicated a good response to treatment and favourable long-term prognosis. Lifetime maintenance therapy is most often required.
authors.affiliation: Department of Medicine, Sections for Hepatology and Gastroenterology, Umeå University Hospital, Umeå, Sweden. marten.werner@vll.se
authors.forename: Mårten
authors.initials: M
authors.lastname: Werner
authors.forename: Sven
authors.initials: S
authors.lastname: Wallerstedt
authors.forename: Stefan
authors.initials: S
authors.lastname: Lindgren
authors.forename: Sven
authors.initials: S
authors.lastname: Almer
authors.forename: Einar
authors.initials: E
authors.lastname: Björnsson
authors.forename: Annika
authors.initials: A
authors.lastname: Bergquist
authors.forename: Hanne
authors.initials: H
authors.lastname: Prytz
authors.forename: Hanna
authors.initials: H
authors.lastname: Sandberg-Gertzén
authors.forename: Rolf
authors.initials: R
authors.lastname: Hultcrantz
authors.forename: Per
authors.initials: P
authors.lastname: Sangfelt
authors.forename: Ola
authors.initials: O
authors.lastname: Weiland
authors.forename: Bodil
authors.initials: B
authors.lastname: Ohlsson
authors.forename: Ake
authors.initials: A
authors.lastname: Danielsson
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D006801:Humans; D008111:Liver Function Tests; D016031:Liver Transplantation / Q000706:statistics & numerical data; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D012042:Registries; D012307:Risk Factors; D015996:Survival Rate; D013548:Sweden; D016896:Treatment Outcome