Japanese Journal of Rheumatology

, Volume 9, Issue 3, pp 267–272 | Cite as

Thrombotic thrombocytopenic purpura in a patient with a diffuse form of systemic sclerosis

  • Kiyomi Sadamoto
  • Eizo Saito
  • Takehiko Ogawa
  • Hidenari Fukuya
  • Masao Kinoshita
Case Report


This paper presents a patient with a rapidly progressive diffuse form of systemic sclerosis, in whom a clinical course thrombotic thrombocytopenic purpura (TTP) also developed. In spite of treatment with plasmapheresis, predonisolone, aspirin and vincristine, she died of pulmonary bleeding. From the analysis of four other reported cases of scleroderma with TTP and this case, the outcome of TTP seems to be closely related to clinical manifestations. It is suggested that a diffuse form of scleroderma with interstitional manifestations would develop a worse outcome; on the other hand, a limited form without interstitional manifestations has a successful outcome.

Key words

Thrombotic thrombocytopenic purpura systemic sclerosis plasma exchange vincristine 


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Copyright information

© VSP and Japanese Rheumatism Association 1999

Authors and Affiliations

  • Kiyomi Sadamoto
    • 1
  • Eizo Saito
    • 1
  • Takehiko Ogawa
    • 1
  • Hidenari Fukuya
    • 1
  • Masao Kinoshita
    • 1
  1. 1.Fourth Department of Medicine, School of MedicineToho University 2-17-6Meguro-ku, TokyoJapan

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