title: Primary Sclerosing Cholangitis: Multiple Phenotypes, Multiple Approaches.
abstract: Primary sclerosing cholangitis (PSC) is a heterogeneous, idiopathic, inflammatory disorder frequently associated with inflammatory bowel diseases. PSC patients may be classified into several subphenotypes. Investigations of pediatric, nonwhite, and female PSC patients have revealed distinguishing features. The natural history of PSC is variable in progression with numerous possible clinical outcomes. PSC patients may suffer bacterial cholangitis, cholangiocarcinoma, or colorectal adenocarcinoma. Treatments focusing on bile acid therapy and immunosuppression have not proven beneficial. Interest in PSC and international collaboration has led to improved understanding of the heterogeneity and the genetic structure and introduced possible effective therapeutics.
authors.affiliation: Division of Gastroenterology and Hepatology, University of California Davis School of Medicine, 4150 V Street, PSSB 3500, Sacramento, CA 95817, USA.
authors.forename: Souvik
authors.initials: S
authors.lastname: Sarkar
authors.affiliation: Division of Gastroenterology and Hepatology, University of California Davis School of Medicine, 4150 V Street, PSSB 3500, Sacramento, CA 95817, USA. Electronic address: clbowlus@ucdavis.edu.
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Bowlus
mesh_terms: D001652:Bile Ducts / Q000473:pathology*; D015209:Cholangitis, Sclerosing / Q000145:classification / Q000208:ethnology / Q000209:etiology / Q000473:pathology*; D001651:Cholestasis, Extrahepatic / Q000150:complications*; D002780:Cholestasis, Intrahepatic / Q000150:complications; D019693:Hepatitis, Autoimmune / Q000150:complications; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D015212:Inflammatory Bowel Diseases / Q000150:complications; D010641:Phenotype
chemical_list: D007074:Immunoglobulin G
keywords: Autoimmune hepatitis; Diagnosis; IgG4-related sclerosing cholangitis; Primary sclerosing cholangitis; Treatment