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Internal and Emergency Medicine

, Volume 13, Issue 4, pp 475–489 | Cite as

Management of idiopathic recurrent pericarditis in adults and in children: a role for IL-1 receptor antagonism

  • Antonio Brucato
  • Giacomo Emmi
  • Luca Cantarini
  • Andrea Di Lenarda
  • Marco Gattorno
  • Giuseppe Lopalco
  • Renzo Marcolongo
  • Massimo Imazio
  • Alberto Martini
  • Domenico Prisco
IM - REVIEW

Abstract

Recurrent pericarditis is one of the most frequent pericardial diseases, affecting up to 30% of the patients who have experienced acute pericarditis. While the diagnosis of acute pericarditis is sometime straight forward, its etiology and therapeutic management are still a challenge for physicians. In developed countries, the idiopathic form is the most frequent, and the search for an infectious etiology is almost invariably negative. Nevertheless, since standard treatment with nonsteroidal anti-inflammatory drugs and colchicine is not always able to neutralize pericardial inflammation in recurrent pericarditis, anakinra, an IL-1 receptor antagonist, has been proposed as a possible therapeutic alternative for refractory forms. IL-1 is a cytokine that exerts a pivotal role in innate immunity and in the pathogenesis of some autoimmune diseases, such as rheumatoid arthritis, and in autoinflammatory disorders, as familial Mediterranean fever and cryopyrin-associated periodic syndromes. The successful management of patients with acute idiopathic recurrent pericarditis (IRP) needs a teamwork approach, where cardiologists, rheumatologists, clinical immunologists and internists are involved. In this review, we will discuss the clinical and therapeutical challenges of IRP both in adults and children from a clinical practice standpoint. We will also briefly illustrate the main pathogenic mechanisms of IRP to provide internists and cardiologists with the rationale for approaching the use of anakinra in selected clinical cases.

Keywords

Pericardial diseases Recurrent pericarditis Autoimmune diseases Autoinflammatory diseases Interleukin-1Ra Anakinra 

Notes

Acknowledgements

The authors would like to thank Maria L. Urban, Alessandra Bettiol, Stefano Salvati, Chiara Marvisi and Valerio Maniscalco for their help in preparing tables and figures.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Statement of human and animal rights

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

None.

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Copyright information

© SIMI 2018

Authors and Affiliations

  • Antonio Brucato
    • 1
  • Giacomo Emmi
    • 2
  • Luca Cantarini
    • 3
  • Andrea Di Lenarda
    • 4
  • Marco Gattorno
    • 5
  • Giuseppe Lopalco
    • 6
  • Renzo Marcolongo
    • 7
  • Massimo Imazio
    • 8
  • Alberto Martini
    • 5
  • Domenico Prisco
    • 2
  1. 1.Internal Medicine DivisionOspedale Papa Giovanni XXIIIBergamoItaly
  2. 2.Department of Experimental and Clinical MedicineUniversity of FirenzeFirenzeItaly
  3. 3.Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet’s Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis CenterUniversity of SienaSienaItaly
  4. 4.Cardiovascular CenterTriesteItaly
  5. 5.Unit of Autoinflammatory Diseases and Immunodeficiencies, Clinic of Pediatrics and Rheumatology“G. Gaslini” InstituteGenoaItaly
  6. 6.Rheumatology Unit, Department of Emergency and Organ TransplantationUniversity of BariBariItaly
  7. 7.Clinical Immunology, Department of MedicineAzienda Ospedaliera-UniversitàPaduaItaly
  8. 8.AOU Città della Salute e della Scienza di TorinoUniversity CardiologyTorinoItaly

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