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Internal and Emergency Medicine

, Volume 13, Issue 6, pp 839–844 | Cite as

Recurrent pericarditis: still idiopathic? The pros and cons of a well-honoured term

  • Antonio Brucato
  • Massimo Imazio
  • Paul C. Cremer
  • Yehuda Adler
  • Bernhard Maisch
  • George Lazaros
  • Marco Gattorno
  • Alida L. P. Caforio
  • Renzo Marcolongo
  • Giacomo EmmiEmail author
  • Alberto Martini
  • Allan L. Klein
IM - REVIEW

Abstract

In developed countries, more than 80% of cases of acute pericarditis remain without an established diagnosis after a conventional and standard diagnostic approach. These cases are generally labelled as ‘idiopathic’, i.e. without a known cause. This lack of information is a matter of concern for both patients and clinicians. Some years ago, this term reflected the state of the art of scientific knowledge on the topic. Advances have changed this point of view, in light of available molecular techniques like polymerase chain reaction able to identify viral cardiotropic agents in pericardial fluid and biopsies. Furthermore, the remarkable efficacy of interleukin-1 antagonists, a therapy targeting the innate immune response, suggests clinical and pathogenic similarity between a proportion of patients with idiopathic recurrent pericarditis and classical autoinflammatory diseases. So, it seems useful to discuss the pros and cons of using the term “idiopathic” in light of the new knowledge.

Keywords

Pericarditis Idiopathic recurrent pericarditis Innate immune system Autoinflammatory diseases Anakinra IL-1 antagonists 

Notes

Compliance with ethical standards

Conflict of interest

Antonio Luca Brucato has received research grants from Acarpia, Sobi, and Lilly, and speaker fees from Sobi, Menarini, and Novartis; Massimo Imazio has received an institutional research grant from Acarpia e SOBI. Paul C. Cremer, Yehuda Adler, Bernhard Maisch, and George Lazaros and Alida L.P. Caforio declare that they have no conflict of interest; Alberto Martini has no conflict of interest to declare, since starting from 1 March 2016 he has become the Scientific Director of the G. Gaslini Hospital; therefore, his role does not allow rendering private consultancies resulting in personal income. He has performed consultancy activities on behalf of the Gaslini Institute for the companies listed below. Abbvie, Biogen, Boehringer, Bristol Myers and Squibb, EMD Serono, Janssen, Novartis, Pfizer, and R-Pharm. The money received for these activities are directly transferred to the Gaslini Institute’s bank account; Marco Gattorno has received research grants and speaker fees from Novartis and Sobi; Renzo Marcolongo has received fee for consultancy from SOBI; Giacomo Emmi has received fees for consultancy from SOBI, GSK, and Novartis; Allan L. Klein has received research grant from Kiniksa. 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

For this type of study, formal consent is not required.

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Copyright information

© Società Italiana di Medicina Interna 2018

Authors and Affiliations

  • Antonio Brucato
    • 1
  • Massimo Imazio
    • 2
  • Paul C. Cremer
    • 3
  • Yehuda Adler
    • 4
  • Bernhard Maisch
    • 5
  • George Lazaros
    • 6
  • Marco Gattorno
    • 7
  • Alida L. P. Caforio
    • 8
  • Renzo Marcolongo
    • 9
  • Giacomo Emmi
    • 10
    Email author
  • Alberto Martini
    • 7
    • 11
  • Allan L. Klein
    • 3
  1. 1.Internal MedicineOspedale Papa Giovanni XXIIIBergamoItaly
  2. 2.Cardiovascular and Thoracic Department, AOU Città DELLA Salute e della ScienzaUniversity CardiologyTurinItaly
  3. 3.Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Disease, Heart and Vascular InstituteCleveland ClinicClevelandUSA
  4. 4.The Sackler Faculty of Medicine Tel Aviv UniversityThe Chaim Sheba Medical CenterTel HashomerIsrael
  5. 5.Faculty of MedicinePhilipps University of MarburgMarburgGermany
  6. 6.Department of Cardiology, Hippokration HospitalUniversity of Athens Medical SchoolAthensGreece
  7. 7.Clinic of Pediatrics and Rheumatology, Unit of Autoinflammatory Diseases and Immunodeficiencies“G. Gaslini” InstituteGenoaItaly
  8. 8.Division of Cardiology, Department of Cardiologic, Thoracic and Vascular SciencesUniversity of PadovaPadovaItaly
  9. 9.Clinical Immunology, Department of MedicineAzienda Ospedaliera-UniversitàPaduaItaly
  10. 10.Department of Experimental and Clinical MedicineUniversity of FirenzeFlorenceItaly
  11. 11.“G. Gaslini” Institute, Scientific Direction, University of GenoaGenoaItaly

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