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Primary sclerosing cholangitis (PSC) is a cholestatic liver disease that affects the bile ducts, which can have severe consequences including cirrhosis and cholangiocarcinoma. No effective management strategies exist that can alter the progression of this disease.

This review published in discusses the current management strategies for PSC and identifies areas of future research. PSC affects both children and adults, and 70% to 80% have inflammatory bowel disease (IBD). Therefore, screening the IBD population may be beneficial; however, the best strategy is yet to be elucidated, especially as new imaging modalities are developed.

Furthermore, staging and prognostication of PSC can be accomplished using various non-invasive measurements, such as liver stiffness and serum-based measures, as well as prognostic scoring systems such as the Amsterdam criteria, ANALI score, Revised Mayo Score, and other population-specific scoring systems.

There are currently no effective medical therapies that can prevent the progression of disease. Various medications including ursodeoxycholic acid, vancomycin, nonsteroidal farnesoid X receptor (FXR) agonists, norursodeoxycholic acid, fibrates, immunosuppressants, anti-fibrotic therapies, and statins have been evaluated. Further clinical trials are needed to improve the medications available for PSC.

PSC may also require endoscopic retrograde cholangiopancreatography to address complications that may develop, though more needs to be understood about the best techniques to improve outcomes. Patients with PSC are more likely to develop cholangiocarcinoma; however, due to its low incidence, studies have demonstrated a limited benefit of interval surveillance with labs and imaging. The only effective therapy for PSC is liver transplantation, but the recurrence rate is 30%, so more needs to be understood to reduce recurrence of disease post-liver transplant.

This study highlights the current management strategies for PSC and underscores the importance of continued research in order to best develop improved screening, surveillance, and management strategies for patients with PSC.

Wenly Ruan, MD, is a clinical instructor in the division of pediatric gastroenterology, hepatology, and nutrition at Baylor College of Medicine in Houston, Texas.

You can read an interview with the lead study author here, and the abstract of the study here.