Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
, 1 (4), 221-230

Primary Sclerosing Cholangitis: A Review and Update

Affiliations

Primary Sclerosing Cholangitis: A Review and Update

James H Tabibian et al. Liver Res.

Abstract

Primary sclerosing cholangitis (PSC) is a rare, chronic, cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and cholangiographically by fibro-obliterative inflammation of the bile ducts. In a clinically significant proportion of patients, PSC progresses to cirrhosis, end-stage liver disease, and/or hepatobiliary cancer, though the disease course can be highly variable. Despite clinical trials of numerous pharmacotherapies over several decades, safe and effective medical therapy remains to be established. Liver transplantation is an option for select patients with severe complications of PSC, and its outcomes are generally favorable. Periodic surveillance testing for pre- as well as post-transplant patients is a cornerstone of preventive care and health maintenance. Here we provide an overview of PSC including its epidemiology, etiopathogenesis, clinical features, associated disorders, surveillance, and emerging potential therapies.

Keywords: Bile duct diseases; Cholangiocarcinoma; Cholangiography; Cholestasis; Magnetic resonance imaging; Natural history; Primary sclerosing cholangitis (PSC).

Conflict of interest statement

Conflict of interest The authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1. Typical cholangiographic findings of primary sclerosing cholangitis
Endoscopic retrograde cholangiography showing the typical cholangiographic findings of primary sclerosing cholangitis with multifocal stricturing and dilatation of the bile ducts.
Fig. 2
Fig. 2. Diseases associated with primary sclerosing cholangitis (PSC)
It is worth mentioning that primary sclerosing cholangitis has the strongest association with inflammatory bowel disease (IBD), which is coexistent in approximately 70% of individuals with PSC. Among patients with PSC-IBD, the lifetime risk of colorectal cancer is estimated at 30× that of the general population and 5× that of individuals with IBD only.
Fig. 3
Fig. 3. Cholangiocarcinoma surveillance in patients with primary sclerosing cholangitis
*Assumes clinical stability and absence of new signs or symptoms; serum CA 19-9 and liver biochemistries to be checked along with ERC or MRCP, and cytology and FISH to be checked with ERC. **Consider early referral to transplant center if progressive worsening of serum tests or other signs or symptoms. Abbreviations: CA 19-9, carbohydrate antigen 19-9; CCA, cholangiocarcinoma; ERC, endoscopic retrograde cholangiography; FISH, fluorescence in situ hybridization; MRCP, magnetic resonance cholangiopancreatography; US, ultrasonography.

Similar articles

  • Primary Sclerosing Cholangitis: A Review and Update on Therapeutic Developments
    JH Tabibian et al. Expert Rev Gastroenterol Hepatol 7 (2), 103-14. PMID 23363260. - Review
    Primary sclerosing cholangitis (PSC) is a chronic, cholestatic, idiopathic liver disease characterized by fibro-obliterative inflammation of the hepatic bile ducts. In a …
  • Primary Sclerosing Cholangitis
    HJ Rodriguez et al. Semin Gastrointest Dis 14 (4), 189-98. PMID 14719769. - Review
    Primary sclerosing cholangitis (PSC) is an idiopathic, chronic cholestatic liver disease of uncertain etiopathogenesis commonly associated with inflammatory bowel disease …
  • Challenges of Cholangiocarcinoma Detection in Patients With Primary Sclerosing Cholangitis
    JH Tabibian et al. J Anal Oncol 1 (1), 50-55. PMID 31897266.
    Primary sclerosing cholangitis (PSC) is a chronic, cholestatic, idiopathic liver disease characterized by fibro-obliterative inflammation of the hepatic bile ducts. In a …
  • Endoscopic Management of Primary Sclerosing Cholangitis
    JH Tabibian et al. Expert Rev Gastroenterol Hepatol 12 (7), 693-703. PMID 29883229. - Review
    Primary sclerosing cholangitis (PSC) is a rare but clinically important cholestatic liver disease. Histopathologically and cholangiographically, PSC is characterized by i …
  • Diagnosis and Treatment of Primary Sclerosing Cholangitis
    RH Wiesner et al. Semin Liver Dis 5 (3), 241-53. PMID 3901273. - Review
    PSC is a progressive chronic hepatobiliary disorder of unknown etiology for which no effective medical or surgical therapy now exists. This syndrome occurs most commonly …
See all similar articles

Cited by 4 PubMed Central articles

LinkOut - more resources

Feedback