Jornal de Síndromes Genéticas e Terapia Gênica
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Idiopathic Recurrent Acute Pericarditis: A Cross Talk between Autoimmunity and Autoinflammation

Rigante D, Napodano S, Lucherini OM, Magnotti F, Muscari I, Guerrini S, Galeazzi M, Frediani B, Vitale A and Cantarini L

Idiopathic recurrent acute pericarditis is the most baffling drawback occurring in around 1/3 of patients who have suffered from acute pericarditis or complicating a host of systemic medical conditions. Various autoimmune diseases can involve the pericardium during the acute phase of the disease, even with no specific signs, and different cases of postviral pericarditis display an autoimmune background. In addition, some autoinflammatory disorders might display self-limited pericardial effusions, which are characterized by chronic recurrence. The clinical efficacy of corticosteroids should give support to the autoimmune origin of idiopathic recurrent acute pericarditis, but the dramatic response to interleukin-1 antagonists in patients with steroid-dependent idiopathic recurrent pericarditis should corroborate its autoinflammatory beginnings. The dividing line between the two medical settings is undefined and both autoimmune and autoinflammatory mechanisms should be advocated in the evaluation of this challenging pericardial disease.