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Idiopathic Pericarditis—an Autoinflammatory Disease?

  • Orphan Diseases (B Manger, Section Editor)
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Abstract

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.

Summary

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.

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  1. Department of Medicine V, Amyloidosis Center and Division of Haematology, Oncology and Rheumatology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany

    Norbert Blank & Hanns-Martin Lorenz

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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.

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Blank, N., Lorenz, HM. Idiopathic Pericarditis—an Autoinflammatory Disease?. Curr Rheumatol Rep 21, 18 (2019). https://doi.org/10.1007/s11926-019-0820-2

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