Advances and controversies in the pathogenesis and management of primary sclerosing cholangitis

作者：Eksteen Bertus^*

来源：British Medical Bulletin, 2014, 110(1): 89-98.

DOI：10.1093/bmb/ldu008

• primary sclerosing cholangitis (PSC)
• inflammatory bowel disease (IBD)
• genetics
• macrophage stimulating protein 1 (MST-1)

Primary sclerosing cholangitis (PSC) is a chronic immune-mediated liver disease that results in end-stage liver disease requiring liver transplantation. PSC is closely associated with inflammatory bowel disease (IBD) with 70% of patients with PSC also suffering from IBD. %26lt;br%26gt;Data for this review were obtained from PubMed. %26lt;br%26gt;Historical and genome-wide association studies have established a strong human leukocyte antigen (HLA) linkage to PSC and defined specific haplotypes associated with enhanced PSC risk. Fifteen non-HLA loci have been defined in PSC. %26lt;br%26gt;The biological role of risk loci in PSC and their place in PSC pathogenesis remain speculative but suggest significant interactions with the host microbiome and therapeutic opportunities. %26lt;br%26gt;Genetics provides a platform to systematically target emerging therapies in PSC. %26lt;br%26gt;Linking PSC genotypes with biology and disease phenotypes paves the way for a personalized medicine approach to manage PSC.

• 出版日期2014-6