Idiopathic Pericarditis—an Autoinflammatory Disease?

  • Norbert BlankEmail author
  • Hanns-Martin Lorenz
Orphan Diseases (B Manger, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Orphan Diseases


Purpose of the Review

Idiopathic acute and recurrent pericarditis are rare diseases of unknown origin. Here, we review trigger factors, pathomechanism, and treatment options for acute and recurrent pericarditis.

Recent Findings

Acute pericarditis can be triggered by viral infections, myocardial ischemia, heart catheter interventions, cardiac surgery or seem to occur without any trigger. Earlier reports about viral nucleic acids in the effusion or myocardial autoantibodies in serum were detected only in a minority of patients. The current pathomechanistic concept focuses on the innate immune system. Clinical trials revealed that colchicine and anti-IL1β-targeted medication were effective to control acute and recurrent attacks.


Activation of the innate immune system in pericarditis suggests that autoinflammation contributes to acute and recurrent pericarditis. The efficacy of colchicine and anti-IL1β-targeted medication in clinical trials indicates that acute and recurrent pericarditis should be regarded as an autoinflammatory disease. Therefore, idiopathic pericarditis should be considered as an autoinflammatory disease.


Acute pericarditis Recurrent pericarditis Autoinflammation Inflammasome Interleukin-1β Colchicine 


Compliance with Ethical Standards

Conflict of Interest

Dr. Blank reports grants and personal fees from Novartis, grants and personal fees from SOBI, during the conduct of the study.

Hanns-Martin Lorenz declares no conflicts of interest.

Human and Animal Rights and Informed Consent

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


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Medicine V, Amyloidosis Center and Division of Haematology, Oncology and RheumatologyUniversity of HeidelbergHeidelbergGermany

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