Clinical Rheumatology

, Volume 29, Issue 10, pp 1191–1194

Etanercept-refractory adult-onset Still’s disease with thrombotic thrombocytopenic purpura successfully treated with tocilizumab

  • Keiichi Sumida
  • Yoshifumi Ubara
  • Junichi Hoshino
  • Tatsuya Suwabe
  • Rikako Hiramatsu
  • Eiko Hasegawa
  • Noriko Hayami
  • Masayuki Yamanouchi
  • Naoki Sawa
  • Fumi Takemoto
  • Kenmei Takaichi
Case Report

DOI: 10.1007/s10067-010-1418-2

Cite this article as:
Sumida, K., Ubara, Y., Hoshino, J. et al. Clin Rheumatol (2010) 29: 1191. doi:10.1007/s10067-010-1418-2

Abstract

We report the case of a 69-year-old Japanese woman who presented with thrombotic thrombocytopenic purpura (TTP) which had manifested soon after the onset of adult-onset Still’s disease (AOSD). Her disease was multi-drug resistant. She had undergone treatment with high-dose glucocorticoids, two courses of steroid pulse therapy, and cyclosporine A. The patient initially had a favorable response to the administration of etanercept (an anti-tumor necrosis factor agent) and glucocorticoids. However, her disease became refractory to etanercept after 6 months. Therefore, we administered tocilizumab (a humanized monoclonal anti-IL-6 receptor antibody) which dramatically improved the patient’s refractory AOSD with TTP. This is the first report of an effective treatment for AOSD with TTP using the biological agents. Our report strongly suggests that biological agents, especially a humanized monoclonal anti-IL-6 receptor antibody, may be a new option for a safe and effective treatment of multi-drug-resistant AOSD and TTP associated with AOSD.

Keywords

Adult-onset Still’s diseaseEtanerceptThrombotic thrombocytopenic purpuraTocilizumab

Copyright information

© Clinical Rheumatology 2010

Authors and Affiliations

  • Keiichi Sumida
    • 1
  • Yoshifumi Ubara
    • 1
  • Junichi Hoshino
    • 1
  • Tatsuya Suwabe
    • 1
  • Rikako Hiramatsu
    • 1
  • Eiko Hasegawa
    • 1
  • Noriko Hayami
    • 1
  • Masayuki Yamanouchi
    • 1
  • Naoki Sawa
    • 1
  • Fumi Takemoto
    • 1
  • Kenmei Takaichi
    • 1
  1. 1.Nephrology CenterToranomon HospitalTokyoJapan