Successful treatment of a patient with tumor necrosis factor receptor‐associated periodic syndrome using a half‐dose of etanercept |
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Authors: | Koichi Kusuhara Takayuki Hoshina Mitsumasa Saito Masataka Ishimura Hirosuke Inoue Takahiko Horiuchi Tetsuji Sato Toshiro Hara |
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Affiliation: | 1. Department of Pediatrics, University of Occupational and Environmental Health School of Medicine, Kitakyushu;2. Department of Pediatrics;3. Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan |
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Abstract: | 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. |
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Keywords: | cytokine etanercept tumor necrosis factor receptor‐associated periodic syndrome treatment |
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