An Unexpected Case of Eosinophilic Cholangitis

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When evaluating a patient with biliary stricture one needs to consider both benign and malignant causes. About 10% of biliary stricture are benign. Eosinophilic cholangitis (EC) is an extremely rare benign disorder of the biliary tract caused by a fibrosis and stricture from eosinophilic infiltration. Etiology of the disease remains unclear, and often times patients present with obstructive jaundice. A 23-year-old male presented with worsening abdominal pain, vomiting, 17-pound weight loss and jaundice. He reported having clay colored stools, dark colored urine, and pruritus. Physical exam showed mild epigastric tenderness with negative Murphy's sign. Lab work up revealed: WBC with 22.9% eosinophils, Total bilirubin 18.7 mg/dl, direct bilirubin >10.0 mg/dl, AST 247 U/L, ALT 606 U/L, and alkaline phosphatase 588 U/L. Autoimmune antibodies and viral hepatitis serology were negative. Ultrasound of the right upper quadrants showed mild intra-hepatic and extra-hepatic biliary dilation. MRCP depicted moderate to severe intrahepatic biliary ductal dilation at the level of the porta hepatis with consideration for acute obstruction versus post-inflammatory changes. Further evaluation with ECPR revealed a 25mm stricture in the common hepatic duct, a 10mm sphincterotomy was performed and a 10x80mm plastic stent was diploid. Histopathology showed benign ductal mucosa with subepithelial fibrosis and mixed eosinophils and lymphocytes inflammation. Patient was started on high-dose steroids for three months. Follow up CBC revealed a down trending eosinophil count to 4.1% of total WBC. Repeat ECRP revealed incomplete resolution of the stricture. Patient was referred to hepatobiliary surgery for a Roux-en-Y hepaticojejunostomy. Eosinophilic cholangitis is a rare cause of biliary strictures. Diagnoses of eosinophilic cholangitis is based on histopathological evidence of eosinophilic infiltration with or without peripheral eosinophilia. Treatment is a course of high dose corticosteroids, however there is no specific guidelines on duration and dosing of therapy. Resolution of biliary stenosis is usually seen 3-5 weeks after initiation of corticosteroids; in such cases surgical option should be explored.Figure: ERCP displaying intrahepatic and extra hepatic ductal dilation.