The Many Faces of Primary Sclerosing Cholangitis: Controversy Abounds

Primary sclerosing cholangitis (PSC) is notoriously challenging to manage given its heterogeneity with regard to diagnosis, management, and progression. The lack of disease-modifying therapy and variable rate of onset of cirrhosis, portal hypertension-related decompensating events, jaundice, pruritus, biliary complications, and need for liver transplantation is deeply unsettling to clinicians and patients alike. Recent updated practice guidance by the American Association for the Study of Liver Diseases and the European Association for the Study of the Liver endeavored to highlight some of these challenges. However, these references only briefly address clinical dilemmas that providers face on a daily basis. This review aims to further discuss these controversial topics, including providing insight into the utility of ursodeoxycolic acid, the significance of alkaline phosphatase normalization, when to consider PSC variants and mimickers, and the implications of continuous hepatobiliary malignancy screening. In particular, there has been a growing body of literature raising concern about repeat exposure to gadolinium-containing contrast. Patients with PSC are potentially at risk for large lifetime exposure to gadolinium related to frequent magnetic resonance imaging scans and whether this carries any negative long-term adverse effects remains unknown.

Automated summary

Primary sclerosing cholangitis (PSC) poses challenges in diagnosis, management, and progression due to its heterogeneity. Updated practice guidance by major liver disease associations addresses some of these challenges, but fails to thoroughly discuss daily clinical dilemmas. This review aims to delve into controversial topics including the use of ursodeoxycholic acid, normalization of alkaline phosphatase, consideration of PSC variants and mimickers, and the implications of continuous hepatobiliary malignancy screening. Of particular concern is the potential long-term adverse effects of repeated exposure to gadolinium-containing contrast in PSC patients who undergo frequent magnetic resonance imaging scans.