Primary sclerosing cholangitis: diagnostic and treatment approach

Abstract

Introduction. Primary sclerosing cholangitis (PSC) is a rare liver disease characterized by chronic inflammation and fibrosis causing multifocal biliary strictures, biliary stasis, fibrosis, and liver cirrhosis. Being an autoimmune disease, it affects the intra and extrahepatic ducts but, its pathophysiology remains unclear. Aim of Study: To evaluate the current diagnostic and treatment approaches of primary sclerosing cholangitis. Material and methods. A literature review of articles and guidelines published between 2015 to 2023 from PubMed, Elsevier and Wiley Online Library with the use of the keywords such as primary sclerosing cholangitis, cholangiocarcinoma, treatment approach was used for this study. Results. The studies show that PSC is more associated with Inflammatory Bowel Syndrome. The estimated prevalence is 60-80%. Usually, asymptomatic but clinical manifestations such as abdominal pain, pruritis, jaundice, and fatigue are common. The elevation of serum alkaline phosphatase and gamma-glutamyl transferase values in a cholestatic pattern is the biochemical hallmark of PSC. The gold standard of diagnosis is magnetic resonance cholangiography which reveals large-duct fibrotic strictures of the biliary tree. Antimitochondrial antibody test can exclude primary biliary cholangitis. Ursodeoxycholic acid is the most widely used drug for PSC. PSC progresses to biliary fibrosis and gets complicated by cirrhosis, liver failure and cholangiocarcinoma. Conclusions. The cause of PSC remains unclear and treatment approach is lacking. The current treatment approach is limited to management of symptoms. The progressive nature of the disease can lead to liver cirrhosis and finally end with the need for liver transplantation. However, PSC may recur even after liver transplantation.