Cardiac sarcoidosis – management and prognosis (RCD code: III‑3A.3)

Justyna Błaut‑Jurkowska, Magdalena Kaźnica‑Wiatr, Klaudia Knap, Lidia Tomkiewicz‑Pająk, Piotr Podolec, Maria Olszowska

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Sarcoidosis is a systemic, granulomatous disease of unknown etiology. Cardiac involvement in the course of sarcoidosis occurs in 5% of patients. However, about 25% of patient with systemic/pulmonary sarcoidosis might present with asymptomatic myocardial injury. The main manifestations of cardiac sarcoidosis are conduction abnormalities, ventricular arrhythmias and heart failure. Diagnosis of cardiac sarcoidosis remains a challenge for physicians. Treatment should be introduced at the time of diagnosis to prevent potentially lethal progression of cardiac disease. Steroids are the first drugs of choice. Additional anti‑sarcoidosis agents are used as alternatives or to reduce the steroids dose. Some patients require implantation of a cardioverter‑defibrillator for the prevention of sudden cardiac death. Patients with refractory ventricular tachyarrhythmia or severe, intractable heart failure unresponsive to optimal pharmacotherapy, require heart transplantation. Prognosis in cardiac sarcoidosis is highly variable, dependent on location and severity of heart involvement. This article reviews current diagnostic and therapeutic recommendations for cardiac sarcoidosis. JRCD 2017; 3 (2): 37–43


rare disease; heart failure; arrhythmia, pacemaker; echocardiography


Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ER. Am J Respir Crit Care Med 1999; 160: 736–755.

Dubrey SW, Sharma R, Underwood R, et al. Cardiac sarcoidosis: diagnosis and management. Postgrad Med J 2015; 91: 384–394.

Zipse MM, Sauer WH. Cardiac sarcoidosis and consequent arrhythmias. Card Electrophysiol Clin 2015; 7: 235–249.

Kim JS, Judson MA, Donnino R, et al. Cardiac sarcoidosis. Am Heart J 2009; 157: 9–21.

Baughman RP, Teirstein AS, Judson MA, et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001; 164: 1885–1889.

Birnie DH, Sauer WH, Bogun F, et al. HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Heart Rhythm 2014; 11: 1305–1323.

Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation 1978; 58: 1204–1211.

Isobe M, Tezuka D. Isolated cardiac sarcoidosis: clinical characteristics, diagnosis and treatment. Int J Cardiol 2015; 182: 132–140.

Kandolin R, Lehtonen J, Graner M, et al. Diagnosing isolated cardiac sarcoidosis. J Intern Med 2011; 270: 461–468.

Tezuka D, Terashima M, Kato Y, et al. Clinical characteristics of definite or suspected isolated cardiac sarcoidosis: application of cardiac magnetic resonance imaging and 18F-Fluoro-2-deoxyglucose positron‑emission tomography/ computerized tomography. J Card Fail 2015; 21: 313–322.

Cooper LT, Baughman KL, Feldman AM, et al. The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology Endorsed by the Heart Failure Society of. Eur Heart J 2007; 28: 3076–3093.

Houston BA, Mukherjee M. Cardiac sarcoidosis: clinical manifestations, imaging characteristics, and therapeutic approach. Clin Med Insights Cardiol 2014; 8: 31–37.

Kusano KF, Satomi K. Diagnosis and treatment of cardiac sarcoidosis. Heart 2016; 102: 184–190.

Hamzeh NY, Wamboldt FS, Weinberger HD. Management of cardiac sarcoidosis in the United States: a Delphi study. Chest 2012; 141: 154–162.

Sekhri V, Sanal S, Delorenzo LJ, et al. Cardiac sarcoidosis: a comprehensive review. Arch Med Sci Termedia Publishing 2011; 7: 546–554.

Nunes H, Freynet O, Naggara N, et al. Cardiac sarcoidosis. Semin Respir Crit Care Med 2010; 31: 428–441.

Yazaki Y, Isobe M, Hiroe M, et al. Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. Am J Cardiol 2001; 88: 1006–1010.

Yodogawa K, Seino Y, Shiomura R, et al. Recovery of atrioventricular block following steroid therapy in patients with cardiac sarcoidosis. J Cardiol 2013; 62: 320–325.

Kato Y, Morimoto S, Uemura A, et al. Efficacy of corticosteroids in sarcoidosis presenting with atrioventricular block. Sarcoidosis Vasc Diffuse Lung Dis 2003; 20: 133–137.

Kusano KF. Effect of corticosteroid on arrhythmic events in patients with cardiac sarcoidosis. J Cardiol 2013; 62: 326–327.

Beegle SH, Barba K, Gobunsuy R, et al. Current and emerging pharmacological treatments for sarcoidosis: a review. Drug Des Devel Ther 2013; 7: 325–338.

Vorselaars ADM, Cremers JP, Grutters JC, et al. Cytotoxic agents in sarcoidosis: which one should we choose? Curr Opin Pulm Med 2014; 20: 479–487.

Bussinguer M, Danielian A, Sharma OP. Cardiac sarcoidosis: diagnosis and management. Curr Treat Options Cardiovasc Med 2012; 14: 652–664.

Judson MA, Baughman RP, Costabel U, et al. Efficacy of infliximab in extrapulmonary sarcoidosis: results from a randomised trial. Eur Respir J 2008; 31: 1189–1196.

Crommelin HA, van der Burg LM, Vorselaars ADM, et al. Efficacy of adalimumab in sarcoidosis patients who developed intolerance to infliximab. Respir Med 2016; 115: 72–77.

Sadek MM, Yung D, Birnie DH, et al. Corticosteroid Therapy for Cardiac Sarcoidosis: A Systematic Review. Can J Cardiol. 2013; 29: 1034–1041.

Nagai T, Nagano N, Sugano Y, et al. Effect of Corticosteroid Therapy on Long‑Term Clinical Outcome and Left Ventricular Function in Patients With Cardiac Sarcoidosis. Circ J 2015; 79: 1593–1600.

Bakker JA, Drent M, Bierau J. Relevance of pharmacogenetic aspects of mercaptopurine metabolism in the treatment of interstitial lung disease. Curr Opin Pulm Med 2007; 13: 458–463.

Wijnen PA, Cremers JP, Nelemans PJ, et al. Association of the TNF‑α G‑308A polymorphism with TNF‑inhibitor response in sarcoidosis. Eur Respir J 2014; 43: 1730–1739.

Habersberger J, Manins V, Taylor AJ. Cardiac sarcoidosis. Intern Med J 2008; 38: 270–277.

Brignole M, Auricchio A, Baron‑Esquivias G, et al. 2013 ESC guidelines on cardiac pacing and cardiac resynchronization therapy: the task force on cardiac pacing and resynchronization therapy of the European Society of Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA). Europace 2013; 15; 1070–1118.

Kron J, Ellenbogen KA. Cardiac sarcoidosis: contemporary review. J Cardiovasc Electrophysiol 2015; 26: 104–109.

Lynch JP, Hwang J, Bradfield J, et al. Cardiac involvement in sarcoidosis: evolving concepts in diagnosis and treatment. Semin Respir Crit Care Med 2014;

: 372–390.

Jefic D, Joel B, Good E, et al. Role of radiofrequency catheter ablation of ventricular tachycardia in cardiac sarcoidosis: report from a multicenter registry. Heart Rhythm 2009; 6: 189–195.

Zaidi AR, Zaidi A, Vaitkus PT. Outcome of heart transplantation in patients with sarcoid cardiomyopathy. J Heart Lung Transplant 2007; 26: 714–717.

Burke WM, Keogh A, Maloney PJ, et al. Transmission of sarcoidosis via cardiac transplantation. Lancet 1990; 336:1579.

Yazaki Y, Isobe M, Hiroe M, et al. Prognostic determinants of long‑term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. Am J Cardiol 2001; 88: 1006–1010.



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