Successful treatment of a patient with tumor necrosis factor receptor-associated periodic syndrome using a half-dose of etanercept
Article first published online: 26 JUL 2012
© 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society
Volume 54, Issue 4, pages 552–555, August 2012
How to Cite
Kusuhara, K., Hoshina, T., Saito, M., Ishimura, M., Inoue, H., Horiuchi, T., Sato, T. and Hara, T. (2012), Successful treatment of a patient with tumor necrosis factor receptor-associated periodic syndrome using a half-dose of etanercept. Pediatrics International, 54: 552–555. doi: 10.1111/j.1442-200X.2011.03525.x
- Issue published online: 26 JUL 2012
- Article first published online: 26 JUL 2012
- Received 11 May 2011; revised 31 August 2011; accepted 9 November 2011.
- tumor necrosis factor receptor-associated periodic syndrome;
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.