Gattorno M et al. – Continuous treatment with anakinra effectively controlled both the clinical and laboratory manifestations in patients with tumor necrosis factor receptor-associated periodic syndrome (TRAPS) and prevented disease relapses. Methods
Objective was to evaluate the efficacy and safety of treatment with the IL-1 receptor antagonist anakinra in pts with TRAPS requiring high cumulative doses of steroids
4 children (mean age 9.1 yrs) and 1 adult (age 33 yrs) 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
All pts were treated with anakinra (1.5 mg/kg/day)
Results
All pts had a prompt response to anakinra, with disappearance of symptoms and normalization of acute-phase reactant levels, including serum amyloid A (SAA)
In all pediatric pts, anakinra was withdrawn after 15 days of treatment
After a few days (mean 5.6 days) a disease relapse occurred, which dramatically responded to reintroduction of anakinra
During the following period of observation (mean 11.4 mos), the pts 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
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