Corticosteroid therapy and long-Term outcomes in patients with cardiac sarcoidosis stratified by left ventricular ejection fraction

Aims: This study aimed to investigate the relationship between corticosteroid therapy and long-Term outcomes in patients with cardiac sarcoidosis, stratified by left ventricular ejection fraction (LVEF) at diagnosis. Methods and results: This study conducted a post hoc analysis of the ILLUstration of the Management and prognosIs of JapaNese PATiEnts with Cardiac Sarcoidosis, a retrospective multicentre registry. Cardiac sarcoidosis was diagnosed based on the 2016 Japanese Circulation Society and 2014 Heart Rhythm Society criteria. The primary endpoint was a composite of all-cause death, hospitalization for heart failure, and fatal ventricular arrhythmia events. Patients were divided into three groups based on LVEF: preserved LVEF (≥50%, n = 251), moderately impaired LVEF (LVEF, 35-49%; n = 149), and severely impaired LVEF (<35%, n = 99). Among 499 patients with cardiac sarcoidosis (mean age: 61.6 ± 11.4 years, male: 36.1%), 419 (84.0%) were treated with corticosteroids after diagnosis. During a median follow-up of 33.7 months (interquartile range, 16.8-62.7 months), 144 primary endpoints (28.9%) occurred. Corticosteroid therapy was associated with better prognosis when assessed in terms of primary endpoint in the entire cohort [hazard ratio (HR) 0.61, 95% confidence interval (CI) 0.41-0.89, P = 0.010]. When stratified by LVEF, corticosteroid therapy was significantly associated with a lower incidence of primary endpoints in the preserved LVEF group (HR, 0.30; 95% CI, 0.15-0.57, P < 0.001), but not in the moderately and severely impaired LVEF groups. This association remained robust, even after adjusting for confounders. Conclusion: In this large cohort of cardiac sarcoidosis, corticosteroid therapy was associated with a lower incidence of long-Term outcomes only in patients with preserved LVEF at diagnosis. Clinical Trial Registration: UMIN000034974.