Abstract 13055: Different Impact of Immunosuppressive Therapy on Disease Activity and Cardiac Outcome in Isolated Cardiac Sarcoidosis and Systemic Sarcoidosis

Abstract

Introduction: Sarcoidosis is a systemic granulomatous disease of unknown cause, and cardiac involvement impacts its prognosis. Isolated cardiac sarcoidosis (iCS) has increasingly been recognized; however, its prognosis and efficacy of immunosuppressive therapy remains undetermined. The aim of this study is to clarify the clinical features and outcomes of patients with iCS and systemic sarcoidosis including cardiac involvement (sCS) receiving immunosuppressive therapy. Methods and Results: We retrospectively reviewed the clinical data of 42 patients with sCS and 30 with iCS diagnosed at our institution between 2004 and 2022. We compared the characteristics, and the rate of adverse cardiac events including cardiac death, fatal ventricular tachyarrhythmia, and heart failure hospitalization between groups. The median follow-up duration was 1535 [630, 2555] days, without significant difference between groups. There were no significant differences in sex, NYHA class, and left ventricular ejection fraction between groups. Immunosuppressive agents were administered in 86% of sCS and in 73% of iCS. When analyzed only with patients receiving immunosuppressive therapy (sCS, n=36; iCS, n=21), the cardiac event-free survival was significantly lower in iCS patients than sCS patients (37% vs. 79%, p=0.002). Multivariate analysis showed that iCS was an independent determinant for predicting adverse cardiac events. We evaluated the disease activity in 26 sCS and 16 iCS patients by quantitative measures of FDG-PET including cardiac metabolic volume and total lesion glycolysis, representing accurate three-dimensional distribution and intensity of inflammation. Although iCS patients had lower baseline disease activity than sCS patients, immunosuppressive therapy did not attenuate disease activity in iCS patients in contrast to sCS. Conclusions: iCS showed poorer response to immunosuppressive therapy and worse cardiac prognosis than sCS.