Early primary sclerosing cholangitis – a challenging diagnosis

Abstract

Primary sclerosing cholangitis (PSC) is a rare autoimmune disorder of the biliary system. PSC is fulminant in its course and leads to jaundice and biliary cirrhosis with specific radiological findings of the beading of larger ducts and pruning of smaller ducts. Though cholangiogram is the gold standard for PSC diagnosis, it may be inconclusive in patients with an early disease or small duct PSC or overlap with autoimmune hepatitis. These conditions may require a liver biopsy for confirmation of the underlying pathology. In this case report, we discuss one of these rare instances when a 24-year-old male was admitted with jaundice. During hospitalization, extensive workup, including cholangiograms, remained inconclusive, but based on a high suspicion of PSC, the patient underwent a liver biopsy, which confirmed our diagnoses