PMID- 22884556 OWN - NLM STAT- MEDLINE DCOM- 20130205 LR - 20121001 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 12 IP - 1 DP - 2012 Nov TI - Recurrent pericarditis: autoimmune or autoinflammatory? PG - 60-5 LID - S1568-9972(12)00159-0 [pii] LID - 10.1016/j.autrev.2012.07.023 [doi] AB - Idiopathic recurrent acute pericarditis (IRAP) represents the most troublesome complication of acute pericarditis and occurs in up to 20-50% of patients. It is generally idiopathic or postcardiac injury. IRAP is a disease of suspected immune-mediated pathogenesis. On the other hand, it has been suggested that some of these patients might have an atypical or subclinical form of an autoinflammatory disease, e.g. genetic disorders characterized by primary dysfunction of the innate immune system and caused by mutations of genes involved in the inflammatory response. We found that IRAP patients were negative for mutations associated with familial Mediterranean fever, but 6% (8/131 patients) carry a mutation in the TNFRSF1A gene, encoding the receptor for tumor necrosis factor-alfa. C-reactive protein (CRP) may be useful to follow the disease activity and guide the appropriate length of therapy, with continuation of the attack doses of the drugs until CRP normalization, at which time tapering may be considered. IRAP often needs a multidrug therapy: NSAIDs or aspirin at high dosages every 6-8h, corticosteroids only rarely, at low dosages and with a very gradual tapering (months) and colchicine at low dosages if tolerated. Anakinra could be a solution for patients who do not tolerate other therapies. CI - Copyright (c) 2012 Elsevier B.V. All rights reserved. FAU - Maestroni, Silvia AU - Maestroni S AD - Internal Medicine, Ospedali Riuniti, Bergamo, Italy. smaestroni@ospedaliriuniti.bergamo.it FAU - Di Corato, Paola Rita AU - Di Corato PR FAU - Cumetti, Davide AU - Cumetti D FAU - Chiara, Di Blasi Lo Cuccio AU - Chiara DB FAU - Ghidoni, Silvia AU - Ghidoni S FAU - Prisacaru, Lilia AU - Prisacaru L FAU - Cantarini, Luca AU - Cantarini L FAU - Imazio, Massimo AU - Imazio M FAU - Penco, Silvana AU - Penco S FAU - Pedrotti, Patrizia AU - Pedrotti P FAU - Caforio, Alida L P AU - Caforio AL FAU - Doria, Andrea AU - Doria A FAU - Brucato, Antonio AU - Brucato A LA - eng PT - Journal Article PT - Review DEP - 20120802 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Autoimmune Diseases/genetics/*immunology MH - Humans MH - Inflammation/genetics/*immunology MH - Pericarditis/*drug therapy/genetics/*immunology/pathology MH - Recurrence EDAT- 2012/08/14 06:00 MHDA- 2013/02/06 06:00 CRDT- 2012/08/14 06:00 PHST- 2012/08/14 06:00 [entrez] PHST- 2012/08/14 06:00 [pubmed] PHST- 2013/02/06 06:00 [medline] AID - S1568-9972(12)00159-0 [pii] AID - 10.1016/j.autrev.2012.07.023 [doi] PST - ppublish SO - Autoimmun Rev. 2012 Nov;12(1):60-5. doi: 10.1016/j.autrev.2012.07.023. Epub 2012 Aug 2.