Etanercept-refractory adult-onset Still's disease with thrombotic thrombocytopenic purpura successfully treated with tocilizumab.
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.
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, Tokyo 105-8470, Japan. k_sumida0504@yahoo.co.jp
Keiichi
K
Sumida
Yoshifumi
Y
Ubara
Junichi
J
Hoshino
Tatsuya
T
Suwabe
Rikako
R
Hiramatsu
Eiko
E
Hasegawa
Noriko
N
Hayami
Masayuki
M
Yamanouchi
Naoki
N
Sawa
Fumi
F
Takemoto
Kenmei
K
Takaichi
D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized; D004351:Drug Resistance; D005260:Female; D006801:Humans; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000628:therapy*; D019947:Receptors, Interleukin-6 / Q000037:antagonists & inhibitors; D016706:Still's Disease, Adult-Onset / Q000150:complications / Q000628:therapy*; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D019947:Receptors, Interleukin-6; C502936:tocilizumab