title: Recurrent pericarditis: autoimmune or autoinflammatory?
abstract: 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.
authors.affiliation: Internal Medicine, Ospedali Riuniti, Bergamo, Italy. smaestroni@ospedaliriuniti.bergamo.it
authors.forename: Silvia
authors.initials: S
authors.lastname: Maestroni
authors.forename: Paola Rita
authors.initials: PR
authors.lastname: Di Corato
authors.forename: Davide
authors.initials: D
authors.lastname: Cumetti
authors.forename: Di Blasi Lo Cuccio
authors.initials: DB
authors.lastname: Chiara
authors.forename: Silvia
authors.initials: S
authors.lastname: Ghidoni
authors.forename: Lilia
authors.initials: L
authors.lastname: Prisacaru
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
authors.forename: Massimo
authors.initials: M
authors.lastname: Imazio
authors.forename: Silvana
authors.initials: S
authors.lastname: Penco
authors.forename: Patrizia
authors.initials: P
authors.lastname: Pedrotti
authors.forename: Alida L P
authors.initials: AL
authors.lastname: Caforio
authors.forename: Andrea
authors.initials: A
authors.lastname: Doria
authors.forename: Antonio
authors.initials: A
authors.lastname: Brucato
mesh_terms: D001327:Autoimmune Diseases / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007249:Inflammation / Q000235:genetics / Q000276:immunology*; D010493:Pericarditis / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D012008:Recurrence