Gattorno, M. et al. Persistent efficacy of anakinra in patients with tumor necrosis factor receptor-associated periodic syndrome. Arthritis Rheum. 58, 1516-1520

Istituto Giannina Gaslini, Genoa, Italy.
Arthritis & Rheumatology (Impact Factor: 7.76). 05/2008; 58(5):1516-20. DOI: 10.1002/art.23475
Source: PubMed


To evaluate the efficacy and safety of treatment with the interleukin-1 receptor antagonist anakinra in patients with tumor necrosis factor receptor-associated periodic syndrome (TRAPS) requiring high cumulative doses of steroids.
Four children (mean age 9.1 years [range 4-13 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-phase reactant levels (including serum amyloid A [SAA]). All patients were treated with anakinra (1.5 mg/kg/day).
All of the patients had a prompt response to anakinra, with disappearance of symptoms and normalization of acute-phase 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 In all pediatric patients, anakinra was withdrawn after 15 days of treatment.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 3-8]) a disease relapse occurred, which dramatically responded to reintroduction of anakinra. During the following period of observation (mean 11.4 months [range 4-20 months]), the patients did not experience episodes of fever or other disease-related clinical manifestations. Levels of acute-phase reactants remained in the normal range. No major adverse reactions or severe infections were observed.
Continuous treatment with anakinra effectively controlled both the clinical and laboratory manifestations in patients with TRAPS and prevented disease relapses.

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Available from: Antonella Meini, Dec 22, 2014
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    • "In 2008, the first small cohort of TRAPS patients treated with Anakinra was described (56). Patients were treated with Anakinra 1.5 mg/kg/day. "
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    • "Treatment with anti-IL-1 agents, on the other hand, has been proven to be particularly efficacious in preventing attacks and inducing a rapid and long-lasting remission of the disease, as well as in the prevention and even regression of amyloidosis [77, 80, 83, 84]. "
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    • "Treatment with corticosteroids alleviates the inflammatory symptoms of TRAPS, but does not affect the frequency of attacks. The antitumor necrosis factor inhibitor etanercept has been used with some nonspecific benefit [33], while treatment with IL-1 blockers prevents disease relapse if administered for at least two weeks at the onset of fever [34]. "
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