Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
Hepatic Veno-Occlusive Disease in a Case of Polymyositis Associated with Thrombotic Thrombocytopenic Purpura/hemolytic Uremic Syndrome
Yoh-ichi ISHIDA, Manabu UTIKOSHI, Masao KUROSAKI, Katuroh OHTA, Tatsuya CHUJO, Shou AOYAMA, Kenzo OHSAWA, Katsuhiko SAITO, Hitoshi YOKOYAMA, Satoshi OHTA
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Keywords: endothelium, autoimmune disease, plasma exchange, ascites
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1998 Volume 37 Issue 8 Pages 694-699

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Abstract

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.
(Internal Medicine 37: 694-699, 1998)

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