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Objective:  To describe the role of biotechnological therapies in patients with tumor necrosis factor receptor associated periodic syndrome (TRAPS) and to identify any predictor of complete response.   Methods:  Clinical, laboratory, and therapeutic data from 44 Caucasian TRAPS patients treated with biologic agents were retrospectively collected in 16 Italian tertiary Centers.   Results:  A total of 55 biological courses with anakinra ( n  = 26), canakinumab ( n  = 16), anti-TNF-α agents ( n  = 10), and tocilizumab ( n  = 3) were analyzed. A complete response was observed in 41 (74.5%) cases, a partial response in 9 (16.4%) cases and a treatment failure in 5 (9.1%) cases. The frequency of TRAPS exacerbations was 458.2 flare/100 patients-year during the 12 months prior to the start of biologic treatment and 65.7 flare/100 patients-years during the first 12 months of therapy ( p  < 0.0001). The median duration of attacks was 5.00 (IQR = 10.50) days at the start of biologics and 1.00 (IQR = 0.00) days at the 12-month assessment ( p  < 0.0001). Likewise, a significant reduction was observed in the Autoinflammatory Disease Activity Index during the study period ( p  < 0.0001). A significant corticosteroid sparing effect was observed as early as the first 12 months of treatment both in the number of patients requiring corticosteroids ( p  = 0.025) and in the dosages employed ( p  < 0.0001). A significant reduction was identified in the erythrocyte sedimentation rate ( p  < 0.0001), C reactive protein ( p  < 0.0001), serum amyloid A ( p  < 0.0001), and in the 24-h proteinuria dosage during follow-up ( p  = 0.001). A relapsing-remitting disease course (OR = 0.027, C.I. 0.001–0.841,  p  = 0.040) and the frequency of relapses at the start of biologics (OR = 0.363, C.I. 0.301–0.953,  p  = 0.034) were significantly associated with a complete response. No serious adverse events were observed.   Conclusions:  Treatment with biologic agents is highly effective in controlling clinical and laboratory TRAPS manifestations. Patients with a relapsing-remitting course and a lower frequency of flares at the start of treatment show more likely a complete response to biologic agents.

frontiers in medicine

Biotechnological Agents for Patients With Tumor Necrosis Factor Receptor Associated Periodic Syndrome—Therapeutic Outcome and Predictors of Response: Real-Life Data From the AIDA Network