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

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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.

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Author information

Author notes

  1. Alberto Martini and Allan L. Klein contributed equally.

Authors and Affiliations

  1. Internal Medicine, Ospedale Papa Giovanni XXIII, Bergamo, Italy

    Antonio Brucato

  2. Cardiovascular and Thoracic Department, AOU Città DELLA Salute e della Scienza, University Cardiology, Turin, Italy

    Massimo Imazio

  3. Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Disease, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio, USA

    Paul C. Cremer & Allan L. Klein

  4. The Sackler Faculty of Medicine Tel Aviv University, The Chaim Sheba Medical Center, Tel Hashomer, Israel

    Yehuda Adler

  5. Faculty of Medicine, Philipps University of Marburg, Marburg, Germany

    Bernhard Maisch

  6. Department of Cardiology, Hippokration Hospital, University of Athens Medical School, Athens, Greece

    George Lazaros

  7. Clinic of Pediatrics and Rheumatology, Unit of Autoinflammatory Diseases and Immunodeficiencies, “G. Gaslini” Institute, Genoa, Italy

    Marco Gattorno & Alberto Martini

  8. Division of Cardiology, Department of Cardiologic, Thoracic and Vascular Sciences, University of Padova, Padova, Italy

    Alida L. P. Caforio

  9. Clinical Immunology, Department of Medicine, Azienda Ospedaliera-Università, Padua, Italy

    Renzo Marcolongo

  10. Department of Experimental and Clinical Medicine, University of Firenze, Largo Brambilla 3, 50134, Florence, Italy

    Giacomo Emmi

  11. “G. Gaslini” Institute, Scientific Direction, University of Genoa, Genoa, Italy

    Alberto Martini

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  1. Antonio Brucato
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Correspondence to Giacomo Emmi.

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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.

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Brucato, A., Imazio, M., Cremer, P.C. et al. Recurrent pericarditis: still idiopathic? The pros and cons of a well-honoured term. Intern Emerg Med 13, 839–844 (2018). https://doi.org/10.1007/s11739-018-1907-x

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