Journal
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
Volume 48, Issue 2-3, Pages 263-272Publisher
HUMANA PRESS INC
DOI: 10.1007/s12016-014-8456-5
Keywords
Primary biliary cirrhosis; Ursodeoxycholic acid; Obeticholic acid; Anti-TNF-alpha; CXCL10 monoclonal antibody; Budesonide; Fibrates
Categories
Ask authors/readers for more resources
Primary biliary cirrhosis (PBC) is a rare inflammatory liver disease for which ursodeoxycholic acid (UDCA) is the only therapy approved by the U.S. Food and Drug Administration. Patients with a biochemical response to UDCA therapy have a similar survival rate compared to the general population. However, up to 40 % of PBC patients do not achieve a complete response to UDCA, have an increased risk of liver-related death and liver transplantation, and represent a persistent medical need for new therapies. Several novel drugs have recently been studied and show potential efficacy in PBC. Obeticholic acid, a farnesoid X receptor agonist, has been tested in phase II trials and initial results after 1 year in a phase III international trial suggest that it may be effective in achieving a biochemical response in approximately 40 % of patients who do not completely respond to UDCA. Several small studies on fibrates have suggested that they may have efficacy, but larger studies are needed. Surprisingly, results of immunomodulators and biologics have not yet been able to demonstrate efficacy, but new approaches have shown promise in animal models and their translation to human clinical trials are awaited.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available