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    To analyze, in a cohort of pediatric patients with recurrent pericarditis undergoing anti-interleukin (IL)-1 treatment: the agent and dosing used as first-line treatment, the long-term efficacy of IL-1 blockers, the percentage of patients achieving a drug-free remission, and the presence of variables associated with drug-free remission. Data were collected from patients' charts. The annualized relapse rate (ARR) was used for evaluation of treatment efficacy, and bivariate logistic regression analysis was used for variables associated with drug-free remission. Fifty-eight patients, treated between 2008 and 2018, were included in the study (mean follow-up. 2.6 years). Of the 56 patients treated with first-line drugs, 14 not responsive patients were underdosed. Fifty-seven patients were treated with anakinra: the ARR before and during daily treatment was 3.05 and 0.28, respectively (P < .0001); an increase to 0.83 was observed after the reduction/withdrawal of treatment (P < .0001). The switch from anakinra to canakinumab (5 patients) was associated to an increase of the ARR (0.49 vs 1.46), but without statistical significance (P = .215). At last follow-up, only 9 of the 58 patients had withdrawn all treatments. With the limits of a retrospective study and the heterogeneity between the patients enrolled in the study, a shorter duration of treatment with anakinra was the only variable associated with drug-free remission. This study shows that most pediatric patients with recurrent pericarditis needing IL-1 blockade received an inadequate treatment with first-line agents. The effectiveness of anakinra is supported by this study, but few patients achieved drug-free remission. The different rate of response to anakinra and canakinumab may suggest a possible role of IL-1α in the pathogenesis of recurrent pericarditis. Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.

    Citation

    Roberta Caorsi, Antonella Insalaco, Francesca Bovis, Giorgia Martini, Marco Cattalini, Marcello Chinali, Alessandro Rimini, Chiara Longo, Silvia Federici, Camilla Celani, Giovanni Filocamo, Rita Consolini, Maria Cristina Maggio, Gloria Fadanelli, Francesco Licciardi, Micol Romano, Barbara Lia Teruzzi, Andrea Taddio, Angela Miniaci, Francesco La Torre, Alessandro De Fanti, Giulio Cavalli, Barbara Bigucci, Romina Gallizzi, Matteo Chinello, Massimo Imazio, Antonio Brucato, Rolando Cimaz, Fabrizio De Benedetti, Marco Gattorno. Pediatric Recurrent Pericarditis: Appropriateness of the Standard of Care and Response to IL-1 Blockade. The Journal of pediatrics. 2023 May;256:18-26.e8

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    PMID: 36470465

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