title: Autoimmunity and autoinflammation as the yin and yang of idiopathic recurrent acute pericarditis.
abstract: Autoimmunity and autoinflammation are generally considered as mutually exclusive mechanisms of diseases but may concur to specific syndromes. Idiopathic recurrent acute pericarditis (IRAP) is defined as the recurrence of pericardial symptoms at any point following the prior cessation of acute pericarditis, and the latency is generally 6 weeks. Manifestations of pericarditis such as pericardial friction rub, electrocardiographic changes, and pericardial effusion are less frequent in the subsequent episodes compared to the index attack, and in some cases the only clinical sign is represented by a suggestive chest pain. Several autoimmune diseases may manifest with pericarditis which is often related to viral infections, while postviral pericarditis may in turn display a nonspecific autoimmune background. Similarly, autoinflammatory syndromes such as familial Mediterranean fever and tumor necrosis factor receptor-associated periodic syndrome are characterized by self-limiting pericardial symptoms. Corticosteroids are generally effective, thus supporting the autoimmune nature of IRAP, but dramatic results are obtained with interleukin-1 blocking agents in corticosteroid-dependent cases, pointing to a pathogenic role for the inflammasome. Based on these observations, we submit that IRAP represents a paradigmatic example of the putative coexistence of autoimmunity and autoinflammation: the main aim of this review is to critically discuss the hypothesis as well as the current understanding of this enigmatic clinical condition.
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy. Electronic address: cantariniluca@hotmail.com.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
authors.affiliation: Interdisciplinary Department of Medicine, Rheumatology Unit, Policlinic Hospital, University of Bari, Bari, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lopalco
authors.affiliation: Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milan, Italy; BIOMETRA Department, University of Milan, Milan, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Selmi
authors.affiliation: Institute of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Napodano
authors.affiliation: Institute of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Gabriella
authors.initials: G
authors.lastname: De Rosa
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy; Department of Clinical and Experimental Medicine, University of Padua, Padua, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Caso
authors.affiliation: Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy.
authors.forename: Luisa
authors.initials: L
authors.lastname: Costa
authors.affiliation: Interdisciplinary Department of Medicine, Rheumatology Unit, Policlinic Hospital, University of Bari, Bari, Italy.
authors.forename: Florenzo
authors.initials: F
authors.lastname: Iannone
authors.affiliation: Institute of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
mesh_terms: D000208:Acute Disease; D001327:Autoimmune Diseases / Q000276:immunology; D015551:Autoimmunity; D006801:Humans; D007249:Inflammation / Q000276:immunology; D010493:Pericarditis / Q000276:immunology*; D012008:Recurrence
keywords: Anakinra; Interleukin-1; Pericardial effusion; Recurrence