Abstract

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.

Highlights

  • Acute pericarditis might recur over time and unexpectedly even years after recovery of a typical first attack

  • A cardiac tamponade might emerge as a complication of idiopathic recurrent acute pericarditis (IRAP), and failure to respond to standard treatment should require hospitalization to perform a pericardiocentesis or go ahead with extensive investigation and exclude gout, myxedema, uremia, but mostly primary or secondary neoplasms [3]

  • Diagnosis of pericarditis can be made when chest pain is variably combined with pericardial friction rub, suggestive electrocardiographic changes, new or worsening pericardial effusion, normal creatine kinase-MB, and increased C-reactive protein, but the minimum criteria for diagnosis of IRAP are the combination of typical chest pain, ECG and/or echocardiographic abnormalities, and increased C-reactive protein

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Summary

Introduction

Acute pericarditis might recur over time and unexpectedly even years after recovery of a typical first attack. A cardiac tamponade might emerge as a complication of idiopathic recurrent acute pericarditis (IRAP), and failure to respond to standard treatment should require hospitalization to perform a pericardiocentesis or go ahead with extensive investigation and exclude gout, myxedema, uremia, but mostly primary or secondary neoplasms [3]. Administration of corticosteroids to maintain for at least 4 weeks and slowly taper in the following months is required in different cases of recurrent pericarditis, legitimating the many concerns of both patients and physicians related to steroid-side effects, but corticosteroid therapy is even an independent risk factor for recurrences [7]. Intrapericardial administration of triamcinolone has been attempted in the treatment of recalcitrant forms of IRAP [8]

The Role of Autoimmunity
Autoimmune Diseases
The Role of Autoinflammation
Major clinical finding
ER ER
Findings
Conclusions
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