A Rare Case of Immune Checkpoint Cholangitis

Document Type

Conference Proceeding

Publication Date

10-1-2024

Publication Title

Am J Gastroenterol

Abstract

Introduction: Immune checkpoint inhibitor cholangitis (IMC) due to its rarity poses difficulties in diagnosis and treatment. IMC includes a range of biliary tract injuries with different clinical and pathological characteristics, from small-duct to large-duct involvement. Case Description/Methods: A 32-year-old man presented hospital with presyncope, nausea , and vomiting. Upon computed tomography, he was found to have multiple cryptogenic liver lesions. He had a history of lung adenocarcinoma on maintenance Keytruda. He had multiple admissions related to Keytruda complications which included pancreatitis requiring high-dose steroids, esophagitis, and gastritis (last esophagogastroduodenoscopy showing Severe hemorrhagic gastritis, gastric stenosis). A magnetic resonance cholangiopancreatography was obtained for cholestatic elevation of transaminases and showed intrahepatic and extrahepatic biliary dilatation with periductal enhancement. A liver biopsy was inconclusive. However, the findings could be associated with obstructive changes. The likely differentials were primary versus secondary sclerosing cholangitis. In the setting of prolonged use of pembrolizumab for 1.5 years and taking into consideration the timeline of symptoms, secondary sclerosing cholangitis was diagnosed. She was treated with steroids and keytruda and paclitaxel were discontinued with improvement in symptoms. Discussion: Immune checkpoint inhibitors(ICI) can affect any organ system, including the liver, causing cholangitis, although this is less common than immune-mediated hepatitis. Cholangitis induced by ICIs is categorized into 3 types: small-duct, large-duct, and mixed. Small-duct cholangitis is likely underreported due to the need for liver biopsy for diagnosis. Its pathology includes bile duct loss, minor bile duct injuries, and mixed inflammatory cells, predominantly CD81 T cells, diffuse fibrosis in the extrahepatic bile duct with imaging showing nonobstructive dilatation or stenosis. For treatment, the Barcelona criterion is used, where alkaline phosphatase normalization indicates complete resolution, a 40% decrease suggests a partial response, and less than 40% is unsatisfactory. Steroids alone or with immunosuppression show similar results. It's crucial to differentiate ICI-induced cholangitis from cholangiocarcinoma, which doesn't respond to steroids, and IgG4-related disease, which does. Taxanes can produce a similar picture and may need discontinuation if ICI-related cholangitis is suspected.

Volume

119

Issue

10

First Page

S1711

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