Hepatic veno-occlusive disease in a case of polymyositis associated with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome.

A 50-year-old woman was treated with prednisolone for polymyositis. During the therapy, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS) occurred. Neither plasma infusion nor plasma exchange could relieve the clinical manifestations of TTP/HUS. Moreover, massive ascites appeared and worsened her condition. She died approximately one year after the diagnosis of polymyositis. The autopsy revealed centri-lobular hepatic necrosis and nonthrombotic obliteration of hepatic small veins. The diagnosis of hepatic veno-occlusive disease (VOD) was made. It was suspected that common factors other than cytoreductive therapy had damaged the endothelium and caused TTP/HUS and VOD in a case of polymyositis.