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Autoimmune and Autoinflammatory Pericarditis: Definitions and New Treatments

  • Pericardial Disease (L Klein and CL Jellis, Section Editors)
  • Published:
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Abstract

Purpose of the Review

The purpose of the review is to analyze the pathogenetic mechanisms that underlie acute pericarditis, with attention to autoimmune and autoinflammatory pericarditis, and, in addition, to review the available therapeutic armamentarium.

Recent Findings

Several studies have been published on the use of anti-IL-1 drugs in recurrent pericarditis, including anakinra and rilonacept. The latest, the RHAPSODY study, based on the use of rilonacept in recurrent pericarditis, has recently reached phase 3 with promising results in terms of efficacy and safety.

Summary

Alterations in the function of the inflammasome and the consequent overproduction of IL-1 play a pivotal role in the genesis of autoinflammatory pericarditis. Recent studies added evidence to the importance of anti-IL-1 drugs in the treatment of recurrent pericarditis with raised C-reactive protein. In the era of tailored medicine, anti-IL-1 agents may be very useful in the subset of patients with recurrent pericarditis and a clear inflammatory phenotype.

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  1. Internal Medicine, Fatebefratelli Hospital, Piazzale Principessa Clotilde 3, Milan, Italy

    Emanuele Bizzi, Lucia Trotta, Massimo Pancrazi, Mariangela Nivuori, Valeria Giosia, Luca Matteucci & Daniela Montori

  2. Department of Biomedical and Clinical Sciences, Fatebenefratelli Hospital, Università di Milano, Milan, Italy

    Antonio Brucato

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  1. Emanuele Bizzi
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Drs. Trotta and Pancrazi report grants from Kiniksa. Dr. Brucato reports grants from SOBI, grants from ACARPIA, other from SOBI, and other from KINIKSA. The other authors declare that they have no conflict of interest.

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Bizzi, E., Trotta, L., Pancrazi, M. et al. Autoimmune and Autoinflammatory Pericarditis: Definitions and New Treatments. Curr Cardiol Rep 23, 128 (2021). https://doi.org/10.1007/s11886-021-01549-5

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