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.

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. Clinical, laboratory, and therapeutic data from 44 Caucasian TRAPS patients treated with biologic agents were retrospectively collected in 16 Italian tertiary Centers. A total of 55 biological courses with anakinra ( = 26), canakinumab ( = 16), anti-TNF-α agents ( = 10), and tocilizumab ( = 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 ( < 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 ( < 0.0001). Likewise, a significant reduction was observed in the Autoinflammatory Disease Activity Index during the study period ( < 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 ( = 0.025) and in the dosages employed ( < 0.0001). A significant reduction was identified in the erythrocyte sedimentation rate ( < 0.0001), C reactive protein ( < 0.0001), serum amyloid A ( < 0.0001), and in the 24-h proteinuria dosage during follow-up ( = 0.001). A relapsing-remitting disease course (OR = 0.027, C.I. 0.001-0.841, = 0.040) and the frequency of relapses at the start of biologics (OR = 0.363, C.I. 0.301-0.953, = 0.034) were significantly associated with a complete response. No serious adverse events were observed. 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.