Tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) is one of the representative autoinflammatory diseases characterised by prolonged periodic fever caused by a genetic mutation of the type 1 TNF receptor (TNFR1). Although its molecular pathophysiology mechanism has not been completely elucidated, therapeutic strategies have been developed based on the recent pathological findings, such as the intracellular stress due to deposition of misfolded mutant TNFR1. The effectiveness of the IL-1β inhibitor for TRAPS has been reported, and canakinumab was approved on December 2016 in Japan. We report a case of TRAPS who became secondarily resistant to predonisolone and etanercept, an anti-TNF agent, after eleven years of treatment. Subsequently, canakinumab, a IL-1β inhibitor, was introduced, resulting in prompt improvement of inflammatory symptoms. This is the first case report of Japanese TRAPS patient successfully treated by canakinumab.
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