PMID- 25308531 OWN - NLM STAT- MEDLINE DCOM- 20150518 LR - 20181202 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 14 IP - 2 DP - 2015 Feb TI - Autoimmunity and autoinflammation as the yin and yang of idiopathic recurrent acute pericarditis. PG - 90-7 LID - S1568-9972(14)00217-1 [pii] LID - 10.1016/j.autrev.2014.10.005 [doi] AB - 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. CI - Copyright (c) 2014 Elsevier B.V. All rights reserved. FAU - Cantarini, Luca AU - Cantarini L AD - Research Center of Systemic Autoinflammatory Diseases and Behcet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy. Electronic address: cantariniluca@hotmail.com. FAU - Lopalco, Giuseppe AU - Lopalco G AD - Interdisciplinary Department of Medicine, Rheumatology Unit, Policlinic Hospital, University of Bari, Bari, Italy. FAU - Selmi, Carlo AU - Selmi C AD - Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milan, Italy; BIOMETRA Department, University of Milan, Milan, Italy. FAU - Napodano, Salvatore AU - Napodano S AD - Institute of Pediatrics, Universita Cattolica Sacro Cuore, Rome, Italy. FAU - De Rosa, Gabriella AU - De Rosa G AD - Institute of Pediatrics, Universita Cattolica Sacro Cuore, Rome, Italy. FAU - Caso, Francesco AU - Caso F AD - Research Center of Systemic Autoinflammatory Diseases and Behcet'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. FAU - Costa, Luisa AU - Costa L AD - Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy. FAU - Iannone, Florenzo AU - Iannone F AD - Interdisciplinary Department of Medicine, Rheumatology Unit, Policlinic Hospital, University of Bari, Bari, Italy. FAU - Rigante, Donato AU - Rigante D AD - Institute of Pediatrics, Universita Cattolica Sacro Cuore, Rome, Italy. LA - eng PT - Journal Article PT - Review DEP - 20141012 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Acute Disease MH - Autoimmune Diseases/immunology MH - *Autoimmunity MH - Humans MH - Inflammation/immunology MH - Pericarditis/*immunology MH - Recurrence OTO - NOTNLM OT - Anakinra OT - Interleukin-1 OT - Pericardial effusion OT - Recurrence EDAT- 2014/10/14 06:00 MHDA- 2015/05/20 06:00 CRDT- 2014/10/14 06:00 PHST- 2014/09/05 00:00 [received] PHST- 2014/09/23 00:00 [accepted] PHST- 2014/10/14 06:00 [entrez] PHST- 2014/10/14 06:00 [pubmed] PHST- 2015/05/20 06:00 [medline] AID - S1568-9972(14)00217-1 [pii] AID - 10.1016/j.autrev.2014.10.005 [doi] PST - ppublish SO - Autoimmun Rev. 2015 Feb;14(2):90-7. doi: 10.1016/j.autrev.2014.10.005. Epub 2014 Oct 12.