Novel Pathophysiological, Diagnostic and Therapeutic Concepts in Acute and Recurrent Pericarditis

Aldo Bonaventura; Georgia K Thomas; Michele Golino; Adolfo Gabriele Mauro; Alessandra Vecchié; Marco Giuseppe Del Buono; Stefano Toldo; Nicola Potere; Antonio Abbate

doi:10.31083/j.rcm2403077

Novel Pathophysiological, Diagnostic and Therapeutic Concepts in Acute and Recurrent Pericarditis

Rev Cardiovasc Med. 2023 Mar 3;24(3):77. doi: 10.31083/j.rcm2403077. eCollection 2023 Mar.

Authors

Affiliations

¹ S.C. Medicina Generale 1, Medical Center, Ospedale di Circolo e Fondazione Macchi, ASST Sette Laghi, 21100 Varese, Italy.
² VCU Pauley Heart Center, Virginia Commonwealth University, Richmond, VA 23220, USA.
³ Department of Medicine and Surgery, University of Insubria, 21100 Varese, Italy.
⁴ Department of Cardiovascular Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00161 Rome, Italy.
⁵ Department of Cardiovascular and Pulmonary Sciences, Catholic University of the Sacred Heart, 00161 Rome, Italy.
⁶ Department of Medicine, Division of Cardiovascular Medicine and Robert M. Berne Cardiovascular Research Center, School of Medicine, University of Virginia, Charlottesville, VA 22908, USA.
⁷ Department of Innovative Technologies in Medicine and Dentistry, "G. D'Annunzio" University, 66100 Chieti, Italy.

Abstract

Acute pericarditis is the most frequent pericardial disease characterized by inflammation of the pericardial layers resulting in pain, dyspnea and fatigue. Often limited to an isolated event, up to 30% of patients experience one or more recurrences. There is limited knowledge about the pathophysiology of this disease, possibly due to the limited availability of animal models. More recently, following seminal clinical trials with colchicine and interleukin-1 (IL-1) blockers and a novel murine model of acute pericarditis using zymosan A, it has become clear that the NLRP3 (NACHT, leucine-rich repeat, and pyrin domain-containing protein 3) inflammasome/IL-1 $β$ axis plays a central role in driving acute pericardial inflammation and in sustaining this process during recurrences. Diagnostic management of pericarditis has been implemented with multimodality imaging including echocardiography, cardiac computed tomography, and cardiac magnetic resonance. These imaging modalities provide essential diagnostic and pathogenetic information, and are able to characterize pericardial inflammation, allowing to refine risk stratification and personalize treatment. Recent acquisitions yield relevant implications with regard to the therapeutic management of acute and recurrent pericarditis. Non-steroidal anti-inflammatory drugs (NSAIDs) and colchicine are cornerstone therapies either for acute and recurrent pericarditis. However, the benefits of targeted agents, such as anakinra - a recombinant human IL-1 receptor antagonist - and rilonacept - an IL-1 $α$ /IL-1 $β$ trap, are being increasingly recognized. To this end, phenotyping patients with pericarditis and addressing such therapies to those presenting with auto-inflammatory features (elevated C-reactive protein, sustained pericardial and systemic inflammation, multiple recurrences) is of utmost importance to identify patients who might be more likely to benefit from NLRP3 inflammasome/IL-1 $β$ pathway blockade.

Keywords: IL-1α; IL-1β; NLRP3 inflammasome; acute pericarditis; anakinra; inflammation; recurrent pericarditis; rilonacept.

Publication types

Review