Recurrent Pericarditis in Children and Adolescents

Abstract

Recurrent pericarditis (RP) is a clinical syndrome characterized by recurrent attacks of acute pericardial inflammation. Prognosis quoad vitam is good, although morbidity might be significant, especially in children and adolescents. Multiple potential etiologies result in RP, in the vast majority of cases through autoimmune or autoinflammatory mechanisms. Idiopathic RP is one of the most frequent diagnoses, that requires the exclusion of all known etiologies. Therapeutic advances in the last decade have been significant with the recognition of the effectiveness of anti IL1 therapy, but a correct diagnostic and therapeutic algorithm is of key importance. Unfortunately, most of evidence comes from studies in adult patients. Here we review the etiopathogenesis, diagnosis and management of RP in pediatric patients.

Keywords: adolescents; autoinflammatory diseases; children; myopericarditis; pediatric; pericarditis.

Figure 1

Drivers of pericardial inflammation. Autoimmunity against cardiac antigens as well as dysregulated innate immunity might result in pericardial inflammation. Innate immunity is activated by receptors for pathogen- or damage-associated molecular patterns (PAMPs and DAMPs, respectively). Crucial innate immunity pathways leading to pericardial inflammation depend on inflammasome activity and on TNF receptor-1 (TNFR1). The inflammasome is a multimolecular complex composed of sensor protein such as NLRP3 or pyrin (that self-assemble upon activation), stimuli such as NLRP3 or pyrin, adaptor proteins such as ASC, and pro-caspase-1. Upon inflammasome assembly, pro-caspase 1 releases active caspase 1, which can process pro-IL1 to active IL1. AHA: anti-heart antibodies, AIDA: anti-intercalated disc antibodies.