Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) accounts for 10-20% of all patients with JIA. sJIA should be considered as a polygenic autoinflammatory disease. Interleukin 1 (IL-1) has been shown to be a major mediator of the inflammatory cascade that underlies sJIA. Treatment with anakinra has been reported to be effective in a sizable portion of patients with sJIA.

Objective: To assess clinical response rate and disease course in sJIA patients treated with anakinra. To evaluated whether the response to anakinra was related to baseline variables

Methods: We reviewed 57 (28 F) consecutive patients with sJIA treated with anakinra for at least 6 months in our institution. We analyzed the effect of anakinra on fever, rash, number of active joints, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), white blood cells count, platelets count and ferritin levels. Clinically inactive disease (CID) was defined according to Wallace criteria. Clinical and laboratory data were obtained using a standard data collection form.

Results: The median age at the disease onset was 5.6 (IQR 2.7-10.0) years. The median time from onset to received anakinra was 1.9 (IQR 0.7-9.5) months. At baseline 53/57 (92.9%) of patients had fever and median number of active joints was 2 (IQR 1-4). After 6 months of treatment 40 patients (70.2%) met criteria for inactive disease. Among 57 patients 17 (30.3%) received anakinra in monotherapy and 40 (70.2%) received anakinra with glucocorticoids. There were no statistically significant differences between the two groups for demographic, clinical and laboratory features. 13/17 (76.4%) patients treated with anakinra alone and 27/40 (67.5%) patients treated with anakinra and glucocorticoids met criteria for CID off glucocorticoids at 6 months (p=0.75). Among the 57 patients, 30 (52.6%) received anakinra within 2 months from disease onset. There were no statistically significant differences for demographic, clinical and laboratory features among patients who started anakinra in the first 2 months from disease onset compared to those that started anakinra after 2 months. At 6 months after beginning of anakinra treatment, 28/30 patients (93.3%) who started anakinra within 2 months from disease onset and 12/27 (44.4%) who started anakinra after 2 months from disease onset reached clinical inactive disease off glucocorticoids (p=0.0001). Patients who started anakinra after the first 2 months from disease onset have a significantly higher risk of non-response (OR=8.06, 95% CI: 2.03-32.0).

Conclusion: According with several observations, anakinra is effective in a significant proportion of patients with sJIA. A possible approach to introduce IL-1 inhibitor, with or without concomitant glucocorticoids, early in the disease course taking advantage of a “window of opportunity” has been suggested. Our observation confirms that earlier treatment with anakinra is associated with a better short-term outcome. Moreover, our results show that beginning of treatment after two months of disease is correlated with a high risk of non-response.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/early-treatment-with-anakinra-in-systemic-juvenile-idiopathic-arthritis/