AUTHOR=Vitale Antonio , Obici Laura , Cattalini Marco , Lopalco Giuseppe , Merlini Giampaolo , Ricco Nicola , Soriano Alessandra , La Torre Francesco , Verrecchia Elena , Insalaco Antonella , Dagna Lorenzo , Jaber Masen Abdel , Montin Davide , Emmi Giacomo , Ciarcia Luisa , Barneschi Sara , Parronchi Paola , Ruscitti Piero , Maggio Maria Cristina , Viapiana Ombretta , Sota Jurgen , Gaggiano Carla , Giacomelli Roberto , Sicignano Ludovico Luca , Manna Raffaele , Renieri Alessandra , Lo Rizzo Caterina , Frediani Bruno , Rigante Donato , Cantarini Luca 

TITLE=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

JOURNAL=Frontiers in Medicine

VOLUME=8

YEAR=2021

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2021.668173

DOI=10.3389/fmed.2021.668173

ISSN=2296-858X

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