S2550 Liver Transplantation in a Patient With Refractory Pruritus From Follicular Cholangitis

Abstract

INTRODUCTION: Follicular cholangitis is rare and can present as a mimicker of cholangiocarcinoma. Surgical intervention is considered on follicular cholangitis patients for suspicion of rapidly progressive cholangiocarcinoma. Here we present the case of a 43-year-old female who initially underwent a Whipple procedure due to concern for a cholangiocarcinoma. Pathology showed follicular cholangitis. She continued with intractable pruritus and a liver transplant was pursued for that indication. CASE DESCRIPTION/METHODS: A 43-year-old previously healthy female presented with progressive fatigue and pruritus. Laboratory evaluation was significant for elevated liver chemistries, filamentous-actin of 121 and a positive antinuclear antibody. A liver biopsy showed dense portal inflammation, mostly lymphocytes, rare plasma cells, mild interface hepatitis, paucity of bile ducts, but no florid duct lesion or fibrosis. Initially, she was started on immunosuppression with no improvement in labs. A magnetic resonance cholangiopancreatography (MRCP) was obtained to rule out bile duct obstruction. MRCP was significant for common bile duct (CBD) stones. Endoscopic retrograde cholangio-pancreatography (ERCP) showed CBD stricture with cytology concerning for cholangiocarcinoma. At this time, she underwent a Whipple procedure. Surgical pathology was suggestive of follicular cholangitis and no malignant cells were identified. Post operatively, she continued to have pruritus and fatigue. Immunosuppression was resumed with no change in symptoms. Repeat MRCP showed right hepatic lobe strictures, but subsequent ERCP was unsuccessful due to post-whipple anatomy. Percutaneous cholangiogram was pursued and did not show any obvious lesion. She was then trialed on multiple pruritus medications including naltrexone, rifampin and sertraline without any improvement in symptoms as well as numerous side effects. She was ultimately maintained on ursodiol with minimal improvement. Liver transplant was considered due to quality of life concerns from pruritus. At that time her MELD was 6. She recently received a liver transplant, and is now pruritus free for months, maintained on standard immunosuppression. DISCUSSION: Clinicians should consider follicular cholangitis in the differential for obstructive jaundice. Review of the literature showed 11 cases of follicular cholangitis and one liver transplant due to cirrhosis. This is the first reported liver transplant case for refractory pruritus due to follicular cholangitis.