Clinical Rheumatology

, Volume 26, Issue 1, pp 101–104

A case of mixed connective tissue disease complicated with thrombotic thrombocytopenic purpura

  • Takeshi Kuroda
  • Kouki Matsuyama
  • Takeshi Nakatsue
  • Syuuichi Murakami
  • Hisashi Hasegawa
  • Hideaki Nakayama
  • Minoru Sakatsume
  • Mitsuhiro Ueno
  • Masaaki Nakano
  • Fumitake Gejyo
Case Report

DOI: 10.1007/s10067-005-0105-1

Cite this article as:
Kuroda, T., Matsuyama, K., Nakatsue, T. et al. Clin Rheumatol (2007) 26: 101. doi:10.1007/s10067-005-0105-1

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare complication of mixed connective tissue disease (MCTD). In this report, we describe the case of a 73-year-old Japanese woman with MCTD who developed fever, thrombocytopenia, and microangiopathic hemolytic anemia and was diagnosed with MCTD together with TTP. The activity of von Willebrand factor (vWF) cleaving metalloprotease ADAMTS13 was low and considered to have contributed to the disease activity of TTP. The patient died despite intensive treatment of plasma exchange (PEX) and steroid pulse therapy. Autopsy results revealed that the kidneys had platelet and fibrin thrombi, which occluded capillaries and arterioles. These findings were compatible with TTP and the decreased activity of ADAMTS13 was considered to be associated with the disease activity of TTP.

Keywords

Chronic renal failure Coma MCTD TTP vWF-CP 

Copyright information

© Clinical Rheumatology 2006

Authors and Affiliations

  • Takeshi Kuroda
    • 1
  • Kouki Matsuyama
    • 1
  • Takeshi Nakatsue
    • 1
  • Syuuichi Murakami
    • 1
  • Hisashi Hasegawa
    • 1
  • Hideaki Nakayama
    • 1
  • Minoru Sakatsume
    • 1
  • Mitsuhiro Ueno
    • 1
  • Masaaki Nakano
    • 2
  • Fumitake Gejyo
    • 1
  1. 1.Division of Clinical Nephrology and RheumatologyNiigata University Graduate School of Medical and Dental ScienceNiigata CityJapan
  2. 2.Department of Medical Technology, School of Health Sciences, Faculty of MedicineNiigata UniversityNiigata CityJapan

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