The evolution of natural history of primary sclerosing cholangitis
- 1 March 2017
- journal article
- review article
- Published by Ovid Technologies (Wolters Kluwer Health) in Current Opinion in Gastroenterology
- Vol. 33 (2), 71-77
- https://doi.org/10.1097/mog.0000000000000333
Abstract
Purpose of review Primary sclerosing cholangitis (PSC) is a rare, idiopathic biliary disease often with an insidious onset, variable disease course, and premature death related to benign and malignant PSC-related sequelae. This review aims to discuss the epidemiology, clinical variants, and natural history of PSC, incorporating data from recent population-based studies. Recent findings PSC naturally leads to cirrhosis, cholangiocarcinoma, other hepatobiliary malignancies, dominant strictures, hepatic osteodystrophy, and bacterial cholangitis. The incidence of PSC appears to be increasing, the reasons for which are unclear. The time from diagnosis to liver transplant appears to be longer in more recent studies compared with earlier studies, suggesting a better overall prognosis than previously believed. In addition, with an increasing number of patients undergoing liver transplantation for PSC, the frequency of death because of liver failure has decreased, whereas cancer-related deaths have increased among patients with PSC. Summary PSC is a heterogeneous disease with a variety of clinical outcomes, both fatal and nonfatal. The progression of liver fibrosis in an individual patient is difficult to predict and may vary from a relatively benign, nonprogressive form to a rapidly progressive form with the need for liver transplantation.Keywords
This publication has 57 references indexed in Scilit:
- Cholangiocarcinoma and dominant strictures in patients with primary sclerosing cholangitisEuropean Journal of Gastroenterology & Hepatology, 2012
- The prevalence, incidence and natural history of primary sclerosing cholangitis in an ethnically diverse populationBMC Gastroenterology, 2011
- Bone Disease in Patients With Primary Sclerosing CholangitisGastroenterology, 2011
- Alkaline phosphatase normalization is associated with better prognosis in primary sclerosing cholangitisDigestive and Liver Disease, 2011
- Primary sclerosing cholangitis in genetically diverse populations listed for liver transplantation: Unique clinical and human leukocyte antigen associationsLiver Transplantation, 2010
- Characteristics and clinical course of primary sclerosing cholangitis in France: a prospective cohort studyEuropean Journal of Gastroenterology & Hepatology, 2010
- Outcome and prognostic factors of 391 Japanese patients with primary sclerosing cholangitisLiver International, 2008
- Diagnostic Accuracy of Magnetic Resonance and Endoscopic Retrograde Cholangiography in Primary Sclerosing CholangitisClinical Gastroenterology and Hepatology, 2006
- Prevalence and predictors of esophageal varices in patients with primary sclerosing cholangitisHepatology, 2004
- Primary sclerosing cholangitis: Clinical and immunopathological review of 21 casesJournal of Gastroenterology and Hepatology, 1990