Document Detail

Factors Associated with Response to Therapy and Outcome of Patients with Primary Biliary Cirrhosis with Features of Autoimmune Hepatitis.
MedLine Citation:
PMID:  24076417     Owner:  NLM     Status:  Publisher    
BACKGROUND & AIMS: For patients with primary biliary cirrhosis (PBC) with features of autoimmune hepatitis (AIH), treatment with ursodeoxycholic acid (UDCA), alone or in combination with immunosuppression, is controversial. Little is known about the factors associated with initial response to therapy or outcome. We performed a retrospective analysis of treatment strategies and factors associated with outcomes of patients with PBC-AIH.
METHODS: We analyzed data from 88 patients with PBC-AIH, from 7 centers in 5 countries. First-line therapies included UDCA alone (n=30) or a combination of UDCA and immunosuppression (n=67).
RESULTS: Of patients who received UDCA alone as the first-line therapy, 37% did not respond to treatment. Severe interface hepatitis was independently associated with lack of response to treatment (P=.024; odds ratio [OR], 0.53; 95% confidence interval [CI], 0.004-0.68). The combination of UDCA and immunosuppression was effective in 73% of patients who had not been previously treated or had not responded to UDCA. The presence of advanced fibrosis was associated with lack of response to the combination of UDCA and immunosuppression (P=.003; OR, 0.13; 95% CI, 0.03-0.48). Second-line immunosuppressive agents (cyclosporine, tacrolimus, and mycophenolate mofetil) led to biochemical remission in 54% of patients who did not respond to initial immunosuppression. Liver transplants were given to 4 patients with PBC-AIH. Five patients died during follow-up (3 from liver-related causes).
CONCLUSION: In a retrospective study of a large cohort of patients with PBC-AIH, UDCA alone did not produce a biochemical response in most patients with severe interface hepatitis; these patients require additional therapy with immunosuppression. Second-line immunosuppressive agents are effective in controlling disease activity in patients that do not respond to conventional immunosuppression.
Ersan Ozaslan; Cumali Efe; Alexandra Heurgue-Berlot; Taylan Kav; Chiara Quarneti; Tugrul Purnak; Luigi Muratori; Yücel Ustündag; Solange Bresson-Hadni; Gérard Thiéfin; Thomas D Schiano; Staffan Wahlin; Paolo Muratori
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-9-25
Journal Detail:
Title:  Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association     Volume:  -     ISSN:  1542-7714     ISO Abbreviation:  Clin. Gastroenterol. Hepatol.     Publication Date:  2013 Sep 
Date Detail:
Created Date:  2013-9-30     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101160775     Medline TA:  Clin Gastroenterol Hepatol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2013 AGA Institute. Published by Elsevier Inc. All rights reserved.
Department of Gastroenterology, Numune Research and Education Hospital, Ankara, Turkey.
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