{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T19:07:32Z","timestamp":1791400052457,"version":"4.3.3"},"reference-count":15,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2008,4,25]],"date-time":"2008-04-25T00:00:00Z","timestamp":1209081600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatism"],"published-print":{"date-parts":[[2008,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To evaluate the efficacy and safety of treatment with the interleukin\u20101 receptor antagonist anakinra in patients with tumor necrosis factor receptor\u2013associated periodic syndrome (TRAPS) requiring high cumulative doses of steroids.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Four children (mean age 9.1 years [range 4\u201313 years]) and 1 adult (age 33 years) with TRAPS were enrolled in the study. The 3 children with cysteine mutations (C52Y, C55Y, C43R) had prolonged and frequent attacks of fever. One child with the R92Q mutation and the adult patient with the C43R mutation displayed a more chronic disease course, with fluctuating, nearly continuous symptoms and persistent elevation of acute\u2010phase reactant levels (including serum amyloid A [SAA]). All patients were treated with anakinra (1.5 mg\/kg\/day).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All of the patients had a prompt response to anakinra, with disappearance of symptoms and normalization of acute\u2010phase reactant levels, including SAA. In all pediatric patients, anakinra was withdrawn after 15 days of treatment. After a few days (mean 5.6 days [range 3\u20138]) a disease relapse occurred, which dramatically responded to reintroduction of anakinra. During the following period of observation (mean 11.4 months [range 4\u201320 months]), the patients did not experience episodes of fever or other disease\u2010related clinical manifestations. Levels of acute\u2010phase reactants remained in the normal range. No major adverse reactions or severe infections were observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Continuous treatment with anakinra effectively controlled both the clinical and laboratory manifestations in patients with TRAPS and prevented disease relapses.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.23475","type":"journal-article","created":{"date-parts":[[2008,4,25]],"date-time":"2008-04-25T20:18:19Z","timestamp":1209154699000},"page":"1516-1520","source":"Crossref","is-referenced-by-count":258,"title":["Persistent efficacy of anakinra in patients with tumor necrosis factor receptor\u2013associated periodic syndrome"],"prefix":"10.1002","volume":"58","author":[{"given":"M.","family":"Gattorno","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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