Characteristics and long-term outcome of patients with autoimmune hepatitis related to the initial treatment response

Marten Werner, Sven Wallerstedt, Stefan Lindgren, Sven Almer, Einar Bjornsson, Annika Bergquist, Hanne Prytz, Hanna Sandberg-Gertzen, Rolf Hultcrantz, Per Sangfelt, Ola Weiland, Bodil Ohlsson, Ake Danielsson

Research output: Contribution to journalArticlepeer-review


Objectives. 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. Material and 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.
Original languageEnglish
Pages (from-to)457-467
JournalScandinavian Journal of Gastroenterology
Issue number4
Publication statusPublished - 2010

Subject classification (UKÄ)

  • Gastroenterology and Hepatology

Free keywords

  • follow-up
  • cirrhosis
  • Autoimmune hepatitis
  • azathioprine
  • treatment


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