OP0057 EARLY TREATMENT WITH ANAKINRA IN SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS
Authors: Manuela Pardeo, Claudia Bracaglia, Anna Tulone, Antonella Insalaco, Giulia Marucci, Rebecca Nicolai, Virginia Messia, Emanuela Sacco, Fabrizio De Benedetti
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Systemic juvenile idiopathic arthritis (sJIA) accounts for 10-20% of all patients with JIA. The prominent systemic clinical features, the marked elevation of inflammatory markers and the absence of autoantibodies make this disease different from other JIA forms. 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. <h3>Objectives</h3> 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. <h3>Methods</h3> We reviewed 56 (28 F) consecutive patients with sJIA treated with anakinra for at least 6 months in our institution. The diagnosis of sJIA was established according to the International League of Associations for Rheumatology (ILAR) classification criteria. 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. <h3>Results</h3> The median age at the disease onset was 5.7 (IQR 2.9-10.2) years. The median time from onset to received anakinra was 1.9 (IQR 0.7-9.7) months. At baseline 52/56 (93%) of patients had fever and median number of active joints was 2 (IQR 1-4). After 6 months of treatment 39 patients (69.6%) met criteria for inactive disease. Among 56 patients 17 (30.3%) received anakinra in monotherapy and 39 (69.6%) 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 26/39 (66.6%) patients treated with anakinra and glucocorticoids met criteria for CID off glucocorticoids at 6 months (p=0.54). Among the 56 patients, 29 (51.7%) 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, 27/29 patients (93.1%) 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). <h3>Conclusion</h3> 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. <h3>Reference</h3> [1] Nigrovic PA. Review: is there a window of opportunity for treatment of systemic juvenile idiopathic arthritis? Arthritis Rheumatol 2014;66:1405-13. <h3>Disclosure of Interests</h3> Manuela Pardeo: None declared, Claudia Bracaglia: None declared, Anna Tulone: None declared, Antonella Insalaco: None declared, Giulia Marucci: None declared, Rebecca Nicolai: None declared, Virginia Messia: None declared, Emanuela Sacco: None declared, Fabrizio De Benedetti Grant/research support from: Abbvie, SOBI, Novimmune, Roche, Novartis, Sanofi, Pfizer