2023 Volume 37 Issue 4 Pages 796-802
A woman in her 70s presented to our hospital with loss of appetite and right hypochondrial pain. Blood test results showed a high eosinophil count, a high serum IgG4, and elevated serum levels of hepatobiliary enzymes. Transabdominal, endoscopic, and intraductal ultrasonography revealed diffuse and homogeneous thickening of the common bile duct wall. Contrast-enhanced computed tomography revealed perihilar bile duct stenosis and bilateral supraclavicular, mediastinal, pulmonary hilar, as well as hepatoduodenal ligament, and abdominal para-aortic lymphadenopathy. Based on histopathological evaluation of a bile duct biopsy and a mediastinal lymph node specimen obtained via endoscopic ultrasound-guided fine-needle aspiration, we diagnosed bile duct lesion as eosinophilic cholangiopathy, and thought of the reason of systemic lymphadenopathy as IgG4-related lymphadenopathy. Prednisolone (30mg/day) therapy was initiated, both of them improved.