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Original research
Sex differences in clinical characteristics and prognosis of patients with cardiac sarcoidosis
  1. Takashi Iso1,
  2. Daichi Maeda1,
  3. Yuya Matsue1,
  4. Taishi Dotare1,
  5. Tsutomu Sunayama1,
  6. Kenji Yoshioka2,
  7. Takeru Nabeta3,4,
  8. Yoshihisa Naruse5,
  9. Takeshi Kitai6,
  10. Tatsunori Taniguchi7,
  11. Hidekazu Tanaka8,
  12. Takahiro Okumura9,
  13. Yuichi Baba10,
  14. Tohru Minamino1,11
  1. 1 Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Tokyo, Japan
  2. 2 Department of Cardiology, Kameda Medical Center, Chiba, Japan
  3. 3 Department of Cardiovascular Medicine, Kitasato University School of Medicine, Sagamihara, Japan
  4. 4 Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands
  5. 5 Division of Cardiology, Internal Medicine III, Hamamatsu University School of Medicine, Hamamatsu, Japan
  6. 6 Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan
  7. 7 Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Osaka, Japan
  8. 8 Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe, Japan
  9. 9 Department of Cardiology, Nagoya University Graduate School of Medicine, Nagoya, Japan
  10. 10 Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Nankoku, Japan
  11. 11 Japan Agency for Medical Research and Development-Core Research for Evolutionary Medical Science and Technology (AMED-CREST), Japan Agency for Medical Research and Development, Tokyo, Japan
  1. Correspondence to Dr Yuya Matsue, Department of Cardiovascular Biology and Medicine, Juntendo University School of Medicine Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan; yuya8950{at}; Dr Daichi Maeda, Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan, Bunkyo-ku, Tokyo, Japan; daichimaeda0424{at}


Objective Owing to the paucity of data, this study aimed to investigate sex differences in clinical features and prognosis of patients with cardiac sarcoidosis (CS).

Methods This study was a secondary analysis of the ILLUstration of the Management and prognosIs of JapaNese PATiEnts with Cardiac Sarcoidosis registry—a retrospective multicentre registry that enrolled patients with CS between 2001 and 2017. The primary outcome was potentially fatal ventricular arrhythmia events (pFVAEs)—a composite of sudden cardiac death, sustained ventricular tachycardia lasting >30 s, ventricular fibrillation or the requirement for implantable cardioverter defibrillator therapy.

Results Of the 512 participants (mean age±SD 61.6±11.4 years), 329 (64.2%) were females. Both sexes had peak ages of 60–64 years at diagnosis. Male patients were younger and had a higher prevalence of coronary artery disease and lower left ventricular ejection fraction than female patients. During a median follow-up of 3 years (IQR 1.6–5.6), pFVAEs were observed in 99 patients, with males having a significantly higher risk than females (p=0.002). This association was retained even after adjustment for other risk factors for pFVAEs, including left ventricular ejection fraction (adjusted HR 1.80; 95% CI 1.08 to 3.01, p=0.025).

Conclusion Approximately two-thirds of patients with CS were females, with a peak age of approximately 60 years at clinical diagnosis in both sexes; male patients were younger than female patients. Male patients had a significantly higher risk of pFVAEs than female patients.

Trial registration number UMIN000034974.

  • cardiomyopathies
  • ventricular fibrillation
  • tachycardia, ventricular
  • epidemiology

Data availability statement

Data are available on reasonable request.

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Data availability statement

Data are available on reasonable request.

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  • Twitter @daichi_maeda_3, @yuya_matsue

  • Contributors TI and DM conceived the idea for the study. TD, TS, KY, TN, YN, TK, TT, HT, TO and YB collected the data. DM and YM contributed to the interpretation of the results. TI wrote the manuscript with the assistance of DM and YM. TM supervised the project. YM takes responsibility for the overall content as the guarantor. All authors (1) made substantial contributions to the study concept or the data analysis or interpretation; (2) drafted the manuscript or revised it critically for important intellectual content; (3) approved the final version of the manuscript to be published and (4) agreed to be accountable for all aspects of the work.

  • Funding The ILLUMINATE-CS study was partially supported by Novartis Pharma Research Grants and JSPS KAKENHI (grant number 22K16147).

  • Competing interests YM received an honorarium from Otsuka Pharmaceutical, and Novartis Japan. TO received honoraria from Ono Yakuhin, Otsuka; Novartis and AstraZeneca and research grants from Ono Yakuhin, Amgen Astellas, Pfizer, Alnylam and Alexion (not in connection with the submitted work). HT has received remuneration from AstraZeneca; Otsuka Pharmaceutical; Ono Pharmaceutical; Pfizer; Daiichi Sankyo and Novartis International AG. The other authors have no conflicts of interest to declare.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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