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Risk stratification of patients with cardiac sarcoidosis: the ILLUMINATE-CS registry
Takeru Nabeta1, Takeshi Kitai2, Yoshihisa Naruse3
1Department of Cardiovascular Medicine, Kitasato University School of Medicine, Sagamihara, Japan.
Insights
Cardiac sarcoidosis (CS) patients face significant risks of heart failure and fatal arrhythmias. Key predictors of adverse events include a history of ventricular tachycardia/fibrillation and elevated brain natriuretic peptide levels.
Area of Science:
- Cardiology
- Immunology
- Internal Medicine
Background:
- Cardiac sarcoidosis (CS) is an underdiagnosed condition.
- Understanding the prognosis and risk factors in CS is crucial for patient management.
Purpose of the Study:
- To evaluate the prognosis of patients diagnosed with cardiac sarcoidosis.
- To identify independent prognostic factors for adverse events in CS patients.
Main Methods:
- Retrospective analysis of 512 CS patients diagnosed between 2001 and 2017.
- Primary endpoint: composite of all-cause death, heart failure hospitalization, or fatal ventricular arrhythmia events (FVAE).
- Multivariable Cox regression analysis to identify independent predictors.
Main Results:
- The 10-year estimated event rates were 48.1% for the primary endpoint, 18.0% for all-cause death, 21.1% for heart failure hospitalizations, and 31.9% for FVAE.
- Independent predictors for the primary endpoint included history of ventricular tachycardia/fibrillation (HR 2.53), log-transformed BNP levels (HR 1.28), reduced left ventricular ejection fraction (LVEF) (HR 0.94 per 5% increase), and post-diagnosis ablation for VT (HR 2.65).
Conclusions:
- Despite relatively low mortality, CS patients experience frequent adverse events, primarily FVAE.
- Patients with low LVEF, high BNP, a history of VT/fibrillation, or those requiring ablation for VT are at high risk.
Aims:
This study evaluated the prognosis and prognostic factors of patients with cardiac sarcoidosis (CS), an underdiagnosed disease.
Methods And Results:
Patients from a retrospective multicentre registry, diagnosed with CS between 2001 and 2017 based on the 2016 Japanese Circulation Society or 2014 Heart Rhythm Society criteria, were included. The primary endpoint was a composite of all-cause death, hospitalization for heart failure, and documented fatal ventricular arrhythmia events (FVAE), each constituting exploratory endpoints. Among 512 registered patients, 148 combined events (56 heart failure hospitalizations, 99 documented FVAE, and 49 all-cause deaths) were observed during a median follow-up of 1042 (interquartile range: 518-1917) days. The 10-year estimated event rates for the primary endpoint, all-cause death, heart failure hospitalizations, and FVAE were 48.1, 18.0, 21.1, and 31.9%, respectively. On multivariable Cox regression, a history of ventricular tachycardia (VT) or fibrillation [hazard ratio (HR) 2.53, 95% confidence interval (CI) 1.59-4.00, P < 0.001], log-transformed brain natriuretic peptide (BNP) levels (HR 1.28, 95% CI 1.07-1.53, P = 0.008), left ventricular ejection fraction (LVEF) (HR 0.94 per 5% increase, 95% CI 0.88-1.00, P = 0.046), and post-diagnosis radiofrequency ablation for VT (HR 2.65, 95% CI 1.02-6.86, P = 0.045) independently predicted the primary endpoint.
Conclusion:
Although mortality is relatively low in CS, adverse events are common, mainly due to FVAE. Patients with low LVEF, with high BNP levels, with VT/fibrillation history, and requiring ablation to treat VT are at high risk.
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