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Anakinra treatment in patients with systemic-onset juvenil idiopathic arthritis: “The Valencia Experience”


Treatment with Interleukin-1 (Il-1) receptor antagonist (anakinra) has been reported to be effective in some patients with systemic-onset juvenile idiopathic arthritis (SoJIA).


To examine the safety and the efficacy of anakinra treatment in a regional cohort of SoJIA patients.


We retrospectively reviewed the medical records of patients with SoJIA treated with anakinra (1-3 mg/kg/day) in our unit between December 2004 and july 2010. Anakinra’s effects were studied on several parameters including fever, rash, erythrocyte sedimentation rate, C-reactive protein levels, arthritis joints count, assessment of disease activity (by physician and parent/patient) and pain (by parent/patient). Resulting data were analysed to characterize therapies, clinical course and adverse events.


A total of 22 patients were included. Their mean age at the onset of treatment was 8,6 (1,8-15,6) years; disease duration was 2,4 (0-10,2) years. They had been follow up for 11-56 months. Fever and rash were resolved in 18 patients (82%) within the first 3 months. Active arthritis persisted at 3 months in 22% of patients, at 6 months in 14%, and at 12 months in 22%. Sixteen patients (70%), including 6 patients receiving anakinra as first-line therapy, attained a complete response. One patient stopped anakinra due to severe skin reaction and two patients due to infections: one severe pneumonia and one positive intradermoreaction. We observed 10 episodes of macrophage activation syndrome in 9 patients (40%), 8 episodes were present at the SoJIA diagnosis and 2 while receiving anakinra.


In our experience, first-line therapy with anakinra in SoJIA patients was associated with an improved resolution of symptoms. These results justify further studies of Il-1 inhibition as first-line therapy in SoJIA patients.

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Correspondence to I Marvillet.

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This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Marvillet, I., Penadés, I.C., Montesinos, B.L. et al. Anakinra treatment in patients with systemic-onset juvenil idiopathic arthritis: “The Valencia Experience”. Pediatr Rheumatol 9 (Suppl 1), P71 (2011).

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