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Successful treatment of a patient with tumor necrosis factor receptor‐associated periodic syndrome using a half‐dose of etanercept
Author(s) -
Kusuhara Koichi,
Hoshina Takayuki,
Saito Mitsumasa,
Ishimura Masataka,
Inoue Hirosuke,
Horiuchi Takahiko,
Sato Tetsuji,
Hara Toshiro
Publication year - 2012
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.2011.03525.x
Subject(s) - medicine , etanercept , tumor necrosis factor alpha , tumor necrosis factor receptor , tumor necrosis factors , necrosis , tumor necrosis factor α , oncology
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.