A case of systemic amyloidosis with cholangiectasis diagnosed by liver biopsy

Abstract

A 60-year-old man presented with fever and fatigue in a medical clinic and was given a diagnosis of cholangitis with mild cholangiectasis. The cholangiectasis remained even after treatment with an appropriate antibiotic agent. When the patient was transferred to our hospital for further examination, he was newly suffering from orthostatic hypotension and peripheral facial nerve palsy. Computed tomography (CT) scan revealed multiple low-density areas in the liver and intrahepatic bile duct dilatation. We performed percutaneous liver biopsy, and histopathological findings showed amyloid deposition around the portal vein. We diagnosed his condition as AL amyloidosis. Oral administration of melphalan and dexamethasone improved his clinical features and CT findings. We consider this case as rare in that the deposition of amyloid protein caused cholangiectasis.