Primary sclerosing cholangitis: A review and update

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).

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. 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. 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.