Abstract
Background: Primary Sclerosing Cholangitis (PSC) is a progressive cholangiopathy characterized by multifocal stricturing and fibrosis of the intrahepatic and/or extrahepatic bile ducts. The disease generally follows an indolent course over several years; however, rapidly progressive variants leading to early cirrhosis and liver transplantation are rarely reported, particularly in young woman without associated inflammatory bowel disease (IBD).
Case Presentation: A 23-year-old woman presented with jaundice and pruritus in May 2025. Initial investigations demonstrated cholestatic liver dysfunction with elevated bilirubin and alkaline phosphatase levels. Viral, autoimmune, infectious, and metabolic etiologies were excluded. MRCP revealed multifocal biliary strictures with non-visualization of the common hepatic duct and bilateral hepatic ducts, associated with lobar atrophy and caudate lobe hypertrophy, highly suggestive of primary sclerosing cholangitis. Liver biopsy demonstrated biliary-pattern injury with periductal fibrosis consistent with PSC. Despite treatment with ursodeoxycholic acid and symptomatic therapy, she experienced rapid progression of jaundice and cholestasis within six months. Repeat evaluation revealed worsening of hyperbilirubinemia, coagulopathy, and established biliary cirrhosis. Following multidisciplinary assessment, the patient underwent deceased donor orthotopic liver transplantation with hepaticojejunostomy in December 2025. She remains clinically stable on follow-up. Incidentally, her father had ulcerative colitis for 25 years, developed sigmoid colon cancer in 2020 resulting in a total proctocolectomy with ileal pouch-anal anastomosis (IPAA).
Conclusion: This case highlights an aggressive phenotype of PSC with unusually rapid progression to end-stage liver disease in the absence of IBD. Early recognition of aggressive disease behavior and timely referral for transplantation is essential for favorable outcome.