Advanced

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

Werner, Marten; Wallerstedt, Sven; Lindgren, Stefan LU ; Almer, Sven; Bjornsson, Einar; Bergquist, Annika; Prytz, Hanne LU ; Sandberg-Gertzen, Hanna; Hultcrantz, Rolf and Sangfelt, Per, et al. (2010) In Scandinavian Journal of Gastroenterology 45(4). p.457-467
Abstract
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.... (More)
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. (Less)
Please use this url to cite or link to this publication:
author
, et al. (More)
(Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
follow-up, cirrhosis, Autoimmune hepatitis, azathioprine, treatment
in
Scandinavian Journal of Gastroenterology
volume
45
issue
4
pages
457 - 467
publisher
Taylor & Francis
external identifiers
  • wos:000276960000011
  • scopus:77949884313
ISSN
1502-7708
DOI
10.3109/00365520903555861
language
English
LU publication?
yes
id
38fd5b5a-2ba4-40a3-aa45-7de5b2b93f58 (old id 1601040)
date added to LUP
2010-05-20 10:41:55
date last changed
2018-07-15 03:59:44
@article{38fd5b5a-2ba4-40a3-aa45-7de5b2b93f58,
  abstract     = {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.},
  author       = {Werner, Marten and Wallerstedt, Sven and Lindgren, Stefan and Almer, Sven and Bjornsson, Einar and Bergquist, Annika and Prytz, Hanne and Sandberg-Gertzen, Hanna and Hultcrantz, Rolf and Sangfelt, Per and Weiland, Ola and Ohlsson, Bodil and Danielsson, Ake},
  issn         = {1502-7708},
  keyword      = {follow-up,cirrhosis,Autoimmune hepatitis,azathioprine,treatment},
  language     = {eng},
  number       = {4},
  pages        = {457--467},
  publisher    = {Taylor & Francis},
  series       = {Scandinavian Journal of Gastroenterology},
  title        = {Characteristics and long-term outcome of patients with autoimmune hepatitis related to the initial treatment response},
  url          = {http://dx.doi.org/10.3109/00365520903555861},
  volume       = {45},
  year         = {2010},
}