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Results 51 SOJIA patients (30 F/21 M) with median disease duration of 1.0 years (range 0 days post dx-11.6 years) and median of 4 active joints (range 0-40) at initiation of anakinra were treated with a mean dose of 2.67 mg/kg (range 1-10) with an average follow-up of almost 5 years (range 0.49-9.75) on anakinra. All children had a sJIA signature as previously described (1) by microarray analysis. After 1L-1 blockade, significant improvements were seen in rash (p = 0.0008), fever (p < 0.0001), number of active joints (p = 0.0002), WBC (p < 0.0001), hemoglobin (p < 0.0001), platelets (p < 0.00001), and ESR (p < 0.0001). Twenty-two patients (17 new, 5 with flares) were treated without any steroids on anakinra alone (18), or concomitantly with methotrexate (4). 37/51 (72%) patients met Wallace criteria for inactive disease, 30/51 (59%) for clinical remission on medications, and 13/51 (25%) met criteria for clinical remission. Eight children (16%) had a partial response with important clinical improvements and were able to stop or greatly wean steroids. Five children (10%) had no sustained response. Most side effects were minor although 1 patient had sepsis immediately after IVMP. One patient had a drug induced hepatitis necessitating discontinuation of anakinra. One patient continued anakinra throughout pregnancy and delivered a normal term baby. Four patients with clinical MAS have resolved on anakinra. Gene expression profiling showed a remarkably homogeneous pattern of IL-1 related gene dysregulation (1), which normalized in most patients after anakinra. In some patients there was a time lapse between clinical response and correction of gene expression, suggesting that immune alterations are not completely resolved at time of first clinical response. Most upregulated transcripts encoded innate immunity related proteins. Down regulated transcripts encoded proteins involve in cytoxic/NK cell function and protein synthesis. In a small number of patients, interferon inducible genes were upregulated, especially in patient who had never received steroids and 5/7 of these showed normalization post anakinra.

PReS-FINAL-2178: Clinical and microarray follow-up of SJIA patients treated with anakinra over the past 10 years in a single institution