J Am Heart Assoc. 2021 Sep 2:e021183. doi: 10.1161/JAHA.121.021183. Online ahead of print.
Background Corticosteroid therapy for the treatment of clinically manifest cardiac sarcoidosis is generally recommended. Our group previously systematically reviewed the data in 2013; since then, there has been increasing quality and quantity of data and also interest in nonsteroid agents. Methods and Results Studies were identified from MEDLINE, EMBASE, Cochrane Controlled Trials Register, Cochrane Database of Systematic Reviews, and the National Institutes of Health ClinicalTrials.gov database. The quality of included articles was rated using Scottish Intercollegiate Guidelines Network 50. Outcomes examined were atrioventricular conduction, left ventricular function, ventricular arrhythmias, and mortality. A total of 3527 references were retrieved, and 34 publications met the inclusion criteria. There were no randomized trials, and only 2 studies were rated good quality. In the 34 reports (total of 1297 patients), 1125 patients received corticosteroids, 235 received additional or other immunosuppressant therapy, and 97 patients received no therapy. There were 178 patients treated for atrioventricular conduction disease, with 76/178 (42.7%) improving. In contrast, 21 patients were not treated with corticosteroids and/or immunosuppressant therapy, and none of them improved. Therapy was associated with the prevention of deterioration in left ventricular function. A total of 8 publications reported on ventricular arrhythmia burden, and 19 reported on mortality; the data quality was too limited to draw conclusions for the latter 2 outcomes. Conclusions The best quality data relate to atrioventricular nodal conduction and left ventricular function recovery. In both situations, therapy with corticosteroids and/or immunosuppressant therapy were sometimes associated with positive outcomes. The data quality is too limited to draw conclusions for ventricular arrhythmias and mortality.