title: Advances and controversies in the pathogenesis and management of primary sclerosing cholangitis.
abstract: BACKGROUND
  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.
  
  
  METHODS
  Data for this review were obtained from PubMed.
  
  
  RESULTS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  Genetics provides a platform to systematically target emerging therapies in PSC.
  
  
  CONCLUSIONS
  Linking PSC genotypes with biology and disease phenotypes paves the way for a personalized medicine approach to manage PSC.
authors.affiliation: Division of Gastroenterology and Hepatology, Snyder Institute for Chronic Diseases, University of Calgary, Calgary, AB, Canada b.eksteen@ucalgary.ca.
authors.forename: Bertus
authors.initials: B
authors.lastname: Eksteen
mesh_terms: D000818:Animals; D015209:Cholangitis, Sclerosing / Q000188:drug therapy / Q000209:etiology* / Q000235:genetics; D020022:Genetic Predisposition to Disease; D055106:Genome-Wide Association Study; D006649:Histocompatibility Antigens / Q000235:genetics; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015212:Inflammatory Bowel Diseases / Q000235:genetics; D064307:Microbiota
chemical_list: D006649:Histocompatibility Antigens; D007166:Immunosuppressive Agents
keywords: genetics; inflammatory bowel disease (IBD); macrophage stimulating protein 1 (MST-1); primary sclerosing cholangitis (PSC)