Drug survival of anakinra and canakinumab in monogenic autoinflammatory diseases: observational study from the International AIDA Registry.

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To investigate survival of interleukin (IL)-1 inhibitors in monogenic autoinflammatory disorders (mAID) through drug retention rate (DRR) and identify potential predictive factors of drug survival from a real-life perspective.Multicentre retrospective study analyzing patients affected by the most common mAID treated with anakinra or canakinumab. Survival curves were analyzed with the Kaplan-Meier method. Statistical analysis included a Cox-proportional hazard model to detect factors responsible for drug discontinuation.Seventy-eight patients for a total of 102 treatment regimens were enrolled. The mean treatment duration was 29.59 months. The estimated DRR of IL-1 inhibitors at 12, 24, and 48 months of follow-up was 75.8%, 69.7% and 51.1%, respectively. Patients experiencing an adverse event had a significantly lower DRR (p = 0.019). In contrast, no significant differences were observed between biologic-naïve patients and those previously treated with biologic drugs (p = 0.985) Patients carrying high-penetrance mutations exhibited a significantly higher DRR compared with those with low-penetrance variants (p = 0.015). Adverse events were the only variable associated with a higher hazard of treatment withdrawal (HR 2.573 [CI: 1.223-5.411], p = 0.013) on regression analysis. A significant glucorticoid-sparing effect was observed (p 


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Authors: Jurgen Sota, Donato Rigante, Rolando Cimaz, Marco Cattalini, Micol Frassi, Raffaele Manna, Ludovico Luca Sicignano, Elena Verrecchia, Emma Aragona, Maria Cristina Maggio, Giuseppe Lopalco, Giacomo Emmi, Paola Parronchi, Alberto Cauli, Ewa Wiesik-Szewczyk, José Hernández-Rodríguez, Carla Gaggiano, Maria Tarsia, Mariam Mourabi, Gaafar Ragab, Antonio Vitale, Claudia Fabiani, Bruno Frediani, Vittoria Lamacchia, Alessandra Renieri, Cantarini Luca, Autoinflammatory Diseases Alliance (AIDA) and the Autoinflammatory Diseases Working Group of the Italian Society of Rheumatology (SIR)

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