TY - JOUR
T1 - Primary sclerosing cholangitis and the microbiota
T2 - Current knowledge and perspectives on etiopathogenesis and emerging therapies
AU - Tabibian, James H.
AU - O'Hara, Steven P.
AU - Lindor, Keith
N1 - Funding Information:
This work was supported by National Institutes of Health Grants AI089713 (to SPO) and T32DK007198 (fellowship training for JHT).
PY - 2014/8
Y1 - 2014/8
N2 - Primary sclerosing cholangitis (PSC) is a chronic, fibroinflammatory, cholestatic liver disease of unknown etiopathogenesis. PSC generally progresses to liver cirrhosis, is a major risk factor for hepatobiliary and colonic neoplasia, and confers a median survival to death or liver transplantation of only 12 years. Although it is well recognized that approximately 75% of patients with PSC also have inflammatory bowel disease (IBD), the significance of this association remains elusive. Accumulating evidence now suggests a potentially important role for the intestinal microbiota, and enterohepatic circulation of molecules derived therefrom, as a putative mechanistic link between PSC and IBD and a central pathobiological driver of PSC. In this concise review, we provide a summary of and perspectives regarding the relevant basic, translational, and clinical data, which, taken together, encourage further investigation of the role of the microbiota and microbial metabolites in the etiopathogenesis of PSC and as a potential target for novel pharmacotherapies.
AB - Primary sclerosing cholangitis (PSC) is a chronic, fibroinflammatory, cholestatic liver disease of unknown etiopathogenesis. PSC generally progresses to liver cirrhosis, is a major risk factor for hepatobiliary and colonic neoplasia, and confers a median survival to death or liver transplantation of only 12 years. Although it is well recognized that approximately 75% of patients with PSC also have inflammatory bowel disease (IBD), the significance of this association remains elusive. Accumulating evidence now suggests a potentially important role for the intestinal microbiota, and enterohepatic circulation of molecules derived therefrom, as a putative mechanistic link between PSC and IBD and a central pathobiological driver of PSC. In this concise review, we provide a summary of and perspectives regarding the relevant basic, translational, and clinical data, which, taken together, encourage further investigation of the role of the microbiota and microbial metabolites in the etiopathogenesis of PSC and as a potential target for novel pharmacotherapies.
KW - Cholestatic
KW - Etiopathogenesis
KW - Metabolites
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U2 - 10.3109/00365521.2014.913189
DO - 10.3109/00365521.2014.913189
M3 - Review article
C2 - 24990660
AN - SCOPUS:84904414417
SN - 0036-5521
VL - 49
SP - 901
EP - 908
JO - Scandinavian Journal of Gastroenterology
JF - Scandinavian Journal of Gastroenterology
IS - 8
ER -