Primary Sclerosing Cholangitis in an Elderly Female With Concomitant Gallstone Disease: A Diagnostic Challenge and Literature Review

Primary sclerosing cholangitis (PSC) is a rare cholestatic disease that causes fibrosis and strictures of the bile ducts. It is primarily diagnosed in males between 30 and 40 years of age. Its presentation in elderly females is uncommon and represents a diagnostic challenge. The objective of this study is to present a clinical case of PSC with an atypical presentation in an elderly female with associated cholelithiasis. A 74-year-old female with no medical history presented with jaundice, dark urine, acholia, and pruritus. She was admitted to the emergency department with jaundice and a cholestatic pattern. Initial imaging studies identified cholecystitis and possible extrinsic compression of the bile duct by the gallbladder, mimicking Mirizzi syndrome. Magnetic resonance cholangiopancreatography identified a focal biliary stricture, but serological markers for PSC were negative. Due to persistent pain, a cholecystectomy and liver biopsy were performed. The liver biopsy was positive for PSC. PSC should be considered in elderly patients with persistent cholestatic jaundice despite concomitant gallstone disease. Characteristic cholangiographic findings, together with histopathological evaluation and exclusion of secondary causes, support the diagnosis in atypical presentations.