Ursodeoxycholic acid for cystic fibrosis-related liver disease (Cochrane Review)

A substantive amendment to this systematic review was last made on 01 May 1999. Cochrane reviews are regularly checked and updated if necessary.

Background: Cystic fibrosis-related liver disease peaks in adolescence with up to 20% of people with cystic fibrosis developing chronic liver disease. Early changes in the liver may ultimately result in end stage liver disease with people needing transplantation. One therapeutic option currently used is ursodeoxycholic acid.

Objectives: To assess the effectiveness of ursodeoxycholic acid in cystic fibrosis liver disease.

Search strategy: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group trials register, and contacted drug companies.Date of the most recent search of the Group's register: April 2002.

Selection criteria: Randomised and quasi-randomised trials of use of ursodeoxycholic acid for at least three months compared with placebo or no additional treatment in people with cystic fibrosis.

Data collection and analysis: Two reviewers independently assessed trial eligibility and quality.

Main results: Seven studies were identified, of which three trials involving 118 participants were included. The complex study design used in two trials meant that data could only be analysed for subsets of participants. There was no significant difference in weight change, weighted mean difference -0.90 (95% Confidence Interval -1.94 to 0.14) based on 30 participants from two trials. Improvement in biliary excretion was reported in only one study and no significant change after treatment was shown. Long-term outcomes such as death or need for liver transplantation were not reported.

Reviewers' conclusions: There are few trials assessing the effectiveness of ursodeoxycholic acid. There is insufficient evidence to justify its routine use in cystic fibrosis.

Citation: Cheng K, Ashby D, Smyth R. Ursodeoxycholic acid for cystic fibrosis-related liver disease (Cochrane Review). In: The Cochrane Library, Issue 3, 2003. Oxford: Update Software.

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This is an abstract of a regularly updated, systematic review prepared and maintained by the Cochrane Collaboration. The full text of the review is available in The Cochrane Library (ISSN 1464-780X).

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