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INTRODUCTION: Primary sclerosing cholangitis (PSC) is a progressive cholestatic disorder that ultimately can lead to cirrhosis, liver failure, malignancy and death. It is strongly associated with inflammatory bowel disease (IBD), and though a rare disease, its incidence is increasing. There are no proven medical therapies for PSC. SOURCES OF DATA: Ovid Medline was utilised to search for articles with keywords 'sclerosing cholangitis' and 'cholangiocarcinoma' and containing titles 'primary sclerosing cholangitis', and references of these papers were cross-referenced for further relevant manuscripts. AREAS OF AGREEMENT: PSC is a rare disease, and there is a strong association with risk loci within the major histocompatibility complex and other genes common to other autoimmune diseases. PSC is a premalignant condition, associated with higher rates of hepatobiliary and colorectal cancer in patients with ulcerative colitis (UC). AREAS OF CONTROVERSY: The pathogenesis is unclear, and competing theories exist surrounding toxic bile acids, enhanced homing of particular T cells from the gut to the liver and increased passage of toxins to the liver through a permeable bowel wall. It is unclear whether the higher rate of colonic cancer in PSC/UC occurs in PSC/Crohn's disease. Ursodeoxycholic acid therapy reduces liver enzymes but has not been shown to improve survival. It may reduce the prevalence of bowel cancer. GROWING POINTS: Recent genetic studies have revealed new risk loci, pointing to the importance of the immune system and its interaction with the biome. AREAS TIMELY FOR DEVELOPING RESEARCH: On the basis of the genetic studies discussed earlier, novel agents are being developed and trialled in the treatment of PSC.

Original publication

DOI

10.1093/bmb/ldv019

Type

Journal article

Journal

Br Med Bull

Publication Date

06/2015

Volume

114

Pages

53 - 64

Keywords

CCR9, cholangiocarcinoma, cholestasis, colorectal cancer, genetics, inflammatory bowel disease, primary sclerosing cholangitis, sclerosing cholangitis, ursodeoxycholic acid, Cholangitis, Sclerosing, Digestive System Neoplasms, Hepatitis, Autoimmune, Humans, Immunoglobulin G, Inflammatory Bowel Diseases, Precancerous Conditions, Prognosis