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Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management.

Abstract
Primary sclerosing cholangitis (PSC), first described in the mid-1850s, is a complex liver disease that is heterogeneous in its presentation. PSC is characterized by chronic cholestasis associated with chronic inflammation of the biliary epithelium, resulting in multifocal bile duct strictures that can affect the entire biliary tree. Chronic inflammation leads to fibrosis involving the hepatic parenchyma and biliary tree, which can lead to cirrhosis and malignancy. The etiology of PSC is not fully understood, which in part explains the lack of effective medical therapy for this condition. However, we have begun to better understand the molecular pathogenesis of PSC. The recognition of specific clinical subtypes and their pattern of progression could improve phenotypic and genotypic classification of the disease. We review our current understanding of this enigmatic disorder and discuss important topics for future studies.
AuthorsJohn E Eaton, Jayant A Talwalkar, Konstantinos N Lazaridis, Gregory J Gores, Keith D Lindor
JournalGastroenterology (Gastroenterology) Vol. 145 Issue 3 Pg. 521-36 (Sep 2013) ISSN: 1528-0012 [Electronic] United States
PMID23827861 (Publication Type: Journal Article, Review, Video-Audio Media)
CopyrightCopyright © 2013 AGA Institute. Published by Elsevier Inc. All rights reserved.
Chemical References
  • Cholagogues and Choleretics
  • Immunosuppressive Agents
  • Ursodeoxycholic Acid
Topics
  • Bile Duct Neoplasms (diagnosis, etiology, therapy)
  • Bile Ducts, Intrahepatic
  • Cholagogues and Choleretics (therapeutic use)
  • Cholangiocarcinoma (diagnosis, etiology, therapy)
  • Cholangiopancreatography, Endoscopic Retrograde
  • Cholangitis, Sclerosing (diagnosis, etiology, therapy)
  • Colonic Neoplasms (diagnosis, etiology, therapy)
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Gallbladder Neoplasms (diagnosis, etiology, therapy)
  • Genetic Predisposition to Disease
  • Humans
  • Immunity, Innate
  • Immunosuppressive Agents
  • Intestines (microbiology)
  • Liver Transplantation
  • Metagenome
  • Ursodeoxycholic Acid (therapeutic use)

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