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Management of idiopathic recurrent pericarditis in adults and in children: a role for IL-1 receptor antagonism

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Abstract

Recurrent pericarditis is one of the most frequent pericardial diseases, affecting up to 30% of the patients who have experienced acute pericarditis. While the diagnosis of acute pericarditis is sometime straight forward, its etiology and therapeutic management are still a challenge for physicians. In developed countries, the idiopathic form is the most frequent, and the search for an infectious etiology is almost invariably negative. Nevertheless, since standard treatment with nonsteroidal anti-inflammatory drugs and colchicine is not always able to neutralize pericardial inflammation in recurrent pericarditis, anakinra, an IL-1 receptor antagonist, has been proposed as a possible therapeutic alternative for refractory forms. IL-1 is a cytokine that exerts a pivotal role in innate immunity and in the pathogenesis of some autoimmune diseases, such as rheumatoid arthritis, and in autoinflammatory disorders, as familial Mediterranean fever and cryopyrin-associated periodic syndromes. The successful management of patients with acute idiopathic recurrent pericarditis (IRP) needs a teamwork approach, where cardiologists, rheumatologists, clinical immunologists and internists are involved. In this review, we will discuss the clinical and therapeutical challenges of IRP both in adults and children from a clinical practice standpoint. We will also briefly illustrate the main pathogenic mechanisms of IRP to provide internists and cardiologists with the rationale for approaching the use of anakinra in selected clinical cases.

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Acknowledgements

The authors would like to thank Maria L. Urban, Alessandra Bettiol, Stefano Salvati, Chiara Marvisi and Valerio Maniscalco for their help in preparing tables and figures.

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Authors and Affiliations

  1. Internal Medicine Division, Ospedale Papa Giovanni XXIII, Bergamo, Italy

    Antonio Brucato

  2. Department of Experimental and Clinical Medicine, University of Firenze, Largo Brambilla 3, 50134, Firenze, Italy

    Giacomo Emmi & Domenico Prisco

  3. Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet’s Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy

    Luca Cantarini

  4. Cardiovascular Center, Trieste, Italy

    Andrea Di Lenarda

  5. Unit of Autoinflammatory Diseases and Immunodeficiencies, Clinic of Pediatrics and Rheumatology, “G. Gaslini” Institute, Genoa, Italy

    Marco Gattorno & Alberto Martini

  6. Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy

    Giuseppe Lopalco

  7. Clinical Immunology, Department of Medicine, Azienda Ospedaliera-Università, Padua, Italy

    Renzo Marcolongo

  8. AOU Città della Salute e della Scienza di Torino, University Cardiology, Torino, Italy

    Massimo Imazio

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Brucato, A., Emmi, G., Cantarini, L. et al. Management of idiopathic recurrent pericarditis in adults and in children: a role for IL-1 receptor antagonism. Intern Emerg Med 13, 475–489 (2018). https://doi.org/10.1007/s11739-018-1842-x

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