Successful treatment of a patient with tumor necrosis factor receptor-associated periodic syndrome using a half-dose of etanercept

Koichi Kusuhara, Takayuki Hoshina, Mitsumasa Saito, Masataka Ishimura, Hirosuke Inoue, Takahiko Horiuchi, Tetsuji Sato, Toshiro Hara

研究成果: Contribution to journalArticle査読

6 被引用数 (Scopus)

抄録

TNF receptor-associated periodic syndrome (TRAPS) is caused by mutations of TNFRSF1A gene and characterized by recurrent febrile episodes of prolonged duration and initial good response to steroids. Etanercept, a TNF blocker, has been used as a putative molecular-targeted agent for TRAPS, with some patients showing limited efficacy. Here, we report a patient with TRAPS who recovered from steroid dependency by etanercept and kept remission with a reduced dose of etanercept. The pathophysiology of TRAPS still remains to be elucidated and several hypotheses have been proposed. In the most recent hypothesis, the concerted action of wild-type and mutant TNF receptors plays an important role in provoking enhanced inflammation in TRAPS. The excellent response to etanercept in our patient suggested that there is heterogeneity in TRAPS patients in terms of the contribution of normal TNF signaling to autoinflammation.

本文言語英語
ページ(範囲)552-555
ページ数4
ジャーナルPediatrics International
54
4
DOI
出版ステータス出版済み - 8 2012

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health

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