A Rare Case of Cholangiocarcinoma Complicating C-AncaVasculitis

Abstract

Antineutrophil cytoplasmic antibody (ANCA) - associated vasculitis is a severe autoimmune disorder. Its prognosis has been markedly improved by the introduction of immunosuppressive treatments. Nevertheless, this has been associated with an increased incidence of malignancy. We report the case of a 60-year-old woman who presented C-ANCA vasculitis with renal failure and pulmonary involvement requiring immunosuppressive therapy. A complete
remission was achieved. After a follow-up of 22 months, she presented asthenia with fever and jaundice. The diagnosis of locally advanced peri-hilar cholangiocarcinoma was made. The outcome was unfavorable and the patient died. The incidence of malignancies is increased in ANCA vasculitis. Biliary tumors are uncommon compared to other cancers. The risk is mainly related to exposure to cyclophosphamide.