An unusual case of acute icteric small bile duct disease

The present case refers to an acute icteric cholestatic syndrome of unknown aetiology in a 37-year old man. The underlying biliary disease was of mixed type combining features of diffuse pleomorphic cholangitis and focal sclerosing cholangitis affecting interlobular bile ducts. Our case met the inclusion and exclusion criteria of idiopathic adulthood ductopenia (IAD) regarding sex, age, clinical history and laboratory findings (negative drug history, absence of autoantibodies, no evidence of inflammatory bowel disease, no involvement of large bile ducts, no bacterial or viral infection). The diversity of the histological findings was attributed to different progression stages of the disease. Complete clinical and laboratory improvement after corticosteroid therapy suggests a beneficial effect and points towards an immunemediated process.