Characteristics and long-term outcome of patients with autoimmune hepatitis related to the initial treatment response.
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.
Werner
Mårten
M
Department of Medicine, Sections for Hepatology and Gastroenterology, Umeå University Hospital, Umeå, Sweden. marten.werner@vll.se
Wallerstedt
Sven
S
Lindgren
Stefan
S
Almer
Sven
S
Björnsson
Einar
E
Bergquist
Annika
A
Prytz
Hanne
H
Sandberg-Gertzén
Hanna
H
Hultcrantz
Rolf
R
Sangfelt
Per
P
Weiland
Ola
O
Ohlsson
Bodil
B
Danielsson
Ake
A
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