Introduction: Eosinophilic cholangitis (EC) is a rare benign disorder of the biliary tract. This disorder must be distinguished from Cholangiocarcinoma (CCA) which can be diffi cult since it is characterized by a dense transmural eosinophilic infi ltr...
Introduction: Eosinophilic cholangitis (EC) is a rare benign disorder of the biliary tract. This disorder must be distinguished from Cholangiocarcinoma (CCA) which can be diffi cult since it is characterized by a dense transmural eosinophilic infi ltration of the bile duct. The biliary tree is one of the most frequently affected extrapancreatic organs in autoimmune pancreatitis (AIP), and the term “IgG4-related cholangitis” was recently introduced to refer to the biliary manifestation of IgG4-related systemic disease. In this paper, we discuss an 80-year-old man with EC who was initially diagnosed by hilar CCA or AIP. Case Report: A 80-year-old man presented with epigastric discomfort and nausea. There was no history of foreign travel or signifi cant allergic reactions or medications. Laboratory studies revealed an increased eosinophil count (white blood cell count of 8. 4×103/mm3, with 14. 1% eosinophils). Amylase, lipase and Alkaline phosphatase levels were 208U/L, 21U/L and 114U/L. IgE level was 979. 4 IU/mL. Serum IgG level was elevated to 2260 mg/dL, but serum IgG4 was 369. 3 mg/L. Serology test for parasites were negative. Stool specimens showed no ova or cyst. A computed tomography suggested hilar CCA. A magnetic resonance cholangiopancreatography (MRCP) revealed multifocal irregular stricture in both intrahepatic ducts, common bile duct (CBD) and pancreatic duct (figure 1). Biopsy at papilla and distal CBD revealed no malignant. Biopsies at stomach and duodenum are consistent with Eosinophilic gastroenteritis (figure 2). Bile cytology shows numerous eosinophils and neutrophils. Oral prednisolone and ursodeoxycholic acid were administered. Thereafter, patient`s symptoms were relieved and abnormal laboratory studies were all normalized and MRCP fi ndings showed improvement of irregular stricture. Conclusion: Although the diagnosis of EC is a diffi cult one, biliary stricture is presented with peripheral eosinophilia, EC should be taken into consideration in the differential diagnosis.