Journal
PEDIATRICS INTERNATIONAL
Volume 54, Issue 4, Pages 552-555Publisher
WILEY-BLACKWELL
DOI: 10.1111/j.1442-200X.2011.03525.x
Keywords
cytokine; etanercept; tumor necrosis factor receptor-associated periodic syndrome; treatment
Categories
Funding
- Ministry of Health, Labour and Welfare of Japan
- Ministry of Education, Culture, Sports, Science and Technology of Japan [23591559]
- Grants-in-Aid for Scientific Research [23591559] Funding Source: KAKEN
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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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