IMR Press / RCM / Volume 21 / Issue 3 / DOI: 10.31083/j.rcm.2020.03.102
Open Access Review
Cardiac sarcoidosis: diagnosis and management
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1 Pulmonary Department, Corfu General Hospital, Corfu 49100, Greece
*Correspondence: lefte_mark83@yahoo.gr (Eleftherios Markatis); icpapanikolaou@hotmail.com (Ilias C Papanikolaou)
Rev. Cardiovasc. Med. 2020, 21(3), 321–338; https://doi.org/10.31083/j.rcm.2020.03.102
Submitted: 22 May 2020 | Revised: 16 August 2020 | Accepted: 18 August 2020 | Published: 30 September 2020
(This article belongs to the Special Issue Cardiac fibrosis)
Copyright: © 2020 Markatis et al. Published by IMR Press.
This is an open access article under the CC BY 4.0 license (https://creativecommons.org/licenses/by/4.0/).
Abstract

Sarcoidosis is a chronic inflammatory disease of unknown etiology characterized by multi-organ involvement. End-organ disease consists of granulomatous inflammation, which if left untreated or not resolved spontaneously, leads to permanent fibrosis and end-organ dysfunction. Cardiac involvement and fibrosis in sarcoidosis occur in 5-10% of cases and is becoming increasingly diagnosed. This is due to increased clinical awareness among clinicians and new diagnostic modalities, since magnetic resonance imaging and positron-emission tomography are emerging as “gold standard” tools replacing endomyocardial biopsy. Despite this progress, isolated cardiac sarcoidosis is difficult to differentiate from other causes of arrhythmogenic cardiomyopathy. Cardiac fibrosis leads to congestive heart failure, arrhythmias and sudden cardiac death. Immunosuppressives (mostly corticosteroids) are used for the treatment of cardiac sarcoidosis. Implantable devices like a cardioverter-defibrillator may be warranted in order to prevent sudden cardiac death. In this article current trends in the pathophysiology, diagnosis and management of cardiac sarcoidosis will be reviewed focusing on published research and latest guidelines. Lastly, a management algorithm is proposed.

Keywords
Sarcoidosis
cardiac sarcoidosis
interstitial lung disease
fibrosis
Figures
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