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.
Ishida
Y
Y
Department of Internal Medicine, Toyama City Hospital, Toyama.
Utikoshi
M
M
Kurosaki
M
M
Ohta
K
K
Chujo
T
T
Aoyama
S
S
Ohsawa
K
K
Saito
K
K
Yokoyama
H
H
Ohta
S
S
D000893:Anti-Inflammatory Agents / Q000009:adverse effects; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000150:complications* / Q000209:etiology / Q000628:therapy; D006504:Hepatic Veno-Occlusive Disease / Q000150:complications* / Q000209:etiology / Q000473:pathology; D006801:Humans; D008875:Middle Aged; D010951:Plasma Exchange; D017285:Polymyositis / Q000150:complications* / Q000188:drug therapy; D011239:Prednisolone / Q000009:adverse effects; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000209:etiology / Q000628:therapy
D000893:Anti-Inflammatory Agents; D011239:Prednisolone