University of Cincinnati Cincinnati, OH, United States
Ismaeel Siddiqi, DO, Elizabeth Sprague, MD, Kamran Safdar, MD University of Cincinnati, Cincinnati, OH
Introduction: Secondary sclerosing cholangitis, a cholestatic biliary disease characterized by inflammation and fibrosis leading to the formation of strictures and progressive destruction of the biliary tree, is a rare disorder. This condition is often a result of chronic biliary obstruction, iatrogenic injury, or ischemic cholangiopathy. However, this case discusses an infrequently observed etiology related to immunotherapy because of treatment with PDL-1 inhibitor pembrolizumab.
Case Description/Methods: An 82-year-old woman with urothelial carcinoma who initially underwent neoadjuvant chemotherapy with cisplatin and gemcitabine, followed by cystectomy with recurrence, was started on pembrolizumab. Following her second cycle, she presented to the emergency department with jaundice. CT urogram revealed intrahepatic biliary ductal dilation with subsequent ERCP demonstrating strictures at the hepatic bifurcation. Two biliary stents were placed in the left and right posterior ducts and a single stent in the right anterior duct. Subsequent MRCP revealed irregular left intrahepatic biliary ductal dilation with central biliary stricture at the common hepatic duct bifurcation. Following interventions, she had a continued rise in alkaline phosphatase and total bilirubin. IR cholangiogram was obtained which showed beaded intrahepatic bile ducts predominantly in the central liver. She underwent percutaneous biliary drain placement as well as a liver biopsy, which revealed an acute cholestatic pattern of injury with ductular proliferation and portal neutrophilia consistent with secondary sclerosing cholangitis secondary to pembrolizumab. The patient was started on methylprednisolone 2 mg/kg and mycophenolate mofetil 1,000 mg twice daily. However, she had progressively worsening disease and passed nearly two months following her diagnosis.
Discussion: This case illustrates a rare side effect of PDL-1 inhibitors. Recognition of immunotherapy-induced secondary sclerosing cholangitis is critical to initiate appropriate therapy to prevent the progression of this condition. However, it is also known to be associated with a high incidence of mortality even with aggressive treatment.
Ismaeel Siddiqi indicated no relevant financial relationships.
Elizabeth Sprague indicated no relevant financial relationships.
Kamran Safdar indicated no relevant financial relationships.
Ismaeel Siddiqi, DO, Elizabeth Sprague, MD, Kamran Safdar, MD. P1857 - Secondary Sclerosing Cholangitis With a PDL-1 Checkpoint Inhibitor, Pembrolizumab, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.