Different Impact of Immunosuppressive Therapy on Cardiac Outcomes in Systemic Versus Isolated Cardiac Sarcoidosis

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  • Masunaga Tomoka
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University Division of Cardiovascular Medicine, Research Institute of Angiocardiology, Faculty of Medical Sciences, Kyushu University
  • Hashimoto Toru
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University Division of Cardiovascular Medicine, Research Institute of Angiocardiology, Faculty of Medical Sciences, Kyushu University
  • Fujino Takeo
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University Department of Advanced Cardiopulmonary Failure, Faculty of Medical Sciences, Kyushu University
  • Ohtani Kisho
    Department of Cardiovascular Medicine, National Hospital Organization Kyushu Medical Center
  • Ishikawa Yusuke
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University
  • Yoshitake Tomoaki
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University Division of Cardiovascular Medicine, Research Institute of Angiocardiology, Faculty of Medical Sciences, Kyushu University
  • Shinohara Keisuke
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University
  • Matsushima Shouji
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University Division of Cardiovascular Medicine, Research Institute of Angiocardiology, Faculty of Medical Sciences, Kyushu University
  • Ide Tomomi
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University Division of Cardiovascular Medicine, Research Institute of Angiocardiology, Faculty of Medical Sciences, Kyushu University
  • Yamasaki Yuzo
    Department of Clinical Radiology, Faculty of Medical Sciences, Kyushu University
  • Isoda Takuro
    Department of Clinical Radiology, Faculty of Medical Sciences, Kyushu University
  • Baba Shingo
    Department of Clinical Radiology, Faculty of Medical Sciences, Kyushu University Department of Health Sciences, Graduate School of Medical Sciences, Kyushu University
  • Ishigami Kousei
    Department of Clinical Radiology, Faculty of Medical Sciences, Kyushu University
  • Tsutsui Hiroyuki
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University Division of Cardiovascular Medicine, Research Institute of Angiocardiology, Faculty of Medical Sciences, Kyushu University School of Medicine and Graduate School, International University of Health and Welfare
  • Kinugawa Shintaro
    Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University Division of Cardiovascular Medicine, Research Institute of Angiocardiology, Faculty of Medical Sciences, Kyushu University

Description

<p>Isolated cardiac sarcoidosis (iCS) is increasingly recognized; however, its prognosis and the efficacy of immunosuppressive therapy remain undetermined. We aimed to compare the prognosis of iCS and systemic sarcoidosis including cardiac involvement (sCS) under immunosuppressive therapy.</p><p>We retrospectively reviewed the clinical data of 42 patients with sCS and 30 patients with iCS diagnosed at Kyushu University Hospital from 2004 through 2022. We compared the characteristics and the rate of adverse cardiac events including cardiac death, fatal ventricular tachyarrhythmia, and heart failure hospitalization between the 2 groups. The median follow-up time was 1535 [interquartile range, 630-2555] days, without a significant difference between the groups. There were no significant differences in gender, NYHA class, or left ventricular ejection fraction. Immunosuppressive agents were administered in 86% of sCS and in 73% of iCS patients (P = 0.191). When analyzed only with patients receiving immunosuppressive therapy (sCS, n = 36; iCS, n = 21), the cardiac event-free survival was significantly lower in iCS than sCS (37% versus 79%, P = 0.002). Myocardial LGE content at the initial diagnosis was comparable in both groups. The disease activity was serially evaluated in 26 sCS and 16 iCS patients by quantitative measures of FDG-PET including cardiac metabolic volume and total lesion glycolysis, representing 3-dimensional distribution and intensity of inflammation in the entire heart. Although iCS patients had lower baseline disease activity than sCS patients, immunosuppressive therapy did not attenuate disease activity in iCS in contrast to sCS.</p><p>iCS showed a poorer response to immunosuppressive therapy and a worse cardiac prognosis compared to sCS despite lower baseline disease activity.</p>

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