Primary sclerosing cholangitis (PSC) is a chronic, idiopathic cholangiopathy that can progress to cirrhosis, end-stage liver disease, hepatobiliary cancer, and/or colorectal cancer. The course of PSC is often complicated by portal hypertension, symptoms of cholestasis, and recurrent bacterial cholangitis, among other conditions, with a consequent decrease in survival (median, approximately 20 years) and quality of life. The etiopathogenesis of PSC remains poorly understood, and, as such, pharmacotherapy has yet to be definitively established. Despite its rarity, PSC is the fifth leading indication for liver transplantation (LT) in the United States. Although the only intervention known to extend survival of patients with PSC, LT is costly and invasive, and recurrent PSC affects approximately 30% of LT recipients. Over the past several years, owing in part to progress in the understanding of PSC, novel pharmacotherapeutics have been developed, some of which are currently in the PSC clinical trial pipeline. Here, in the first of a 2-part series, we provide a review and update of the epidemiology, etiopathogenesis, clinical features, and treatment of PSC. The second part of the series will focus on cancer risk, prevention, and surveillance of PSC.
Keywords: Primary sclerosing cholangitis; cirrhosis; colorectal cancer; hepatobiliary cancer; inflammatory bowel disease; liver transplantation; microbiome.
Conflict of interest statement
The authors have no relevant conflicts of interest to disclose.
Primary Sclerosing Cholangitis: A Review and UpdateJH Tabibian et al. Liver Res 1 (4), 221-230. PMID 29977644.Primary sclerosing cholangitis (PSC) is a rare, chronic, cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and …
The Microbiome and Primary Sclerosing CholangitisAH Ali et al. Semin Liver Dis 36 (4), 340-348. PMID 27997974. - ReviewPrimary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with detrimental sequela. In many patients, PSC progresses to end-stage liver disease and hepa …
Primary Sclerosing Cholangitis: Diagnosis, Prognosis, and ManagementS Singh et al. Clin Gastroenterol Hepatol 11 (8), 898-907. PMID 23454027. - ReviewPrimary sclerosing cholangitis (PSC) is a chronic immune-mediated disease of the liver of unclear etiology, characterized by chronic inflammation and fibrosis of bile duc …
Primary Sclerosing Cholangitis: A Clinical UpdateKD Williamson et al. Br Med Bull 114 (1), 53-64. PMID 25981516. - ReviewOn the basis of the genetic studies discussed earlier, novel agents are being developed and trialled in the treatment of PSC.
Primary Sclerosing CholangitisHJ Rodriguez et al. Semin Gastrointest Dis 14 (4), 189-98. PMID 14719769. - ReviewPrimary sclerosing cholangitis (PSC) is an idiopathic, chronic cholestatic liver disease of uncertain etiopathogenesis commonly associated with inflammatory bowel disease …
Cited by 2 PubMed Central articles
Preventative Care in Cholestatic Liver Disease: Pearls for the Specialist and SubspecialistA Malik et al. Liver Res 3 (2), 118-127. PMID 32042471.Cholestatic liver diseases (CLDs) encompass a variety of disorders of abnormal bile formation and/or flow. CLDs often lead to progressive hepatic insult and injury and fo …
Role of Gut Dysbiosis in Liver Diseases: What Have We Learned So Far?H Fukui. Diseases 7 (4). PMID 31726747. - ReviewAccumulating evidence supports that gut dysbiosis may relate to various liver diseases. Alcoholics with high intestinal permeability had a decrease in the abundance of …