Background/Purpose:

• To identify prognostic factors and to assess the effects of immunosuppressive drugs on relapse risk in patients presenting with cardiac sarcoidosis (CS).

Methods:

• From a cohort of 157 patients with CS with a median follow-up of 7 years, we analysed all cardiac and extra-cardiac data and treatments, and assessed relapse-free and overall survival.

Results:

• The 10-year survival rate was 90% (95% CI, 84-96). Baseline factors associated with mortality were the presence of high degree atrioventricular block (HR, 5.56, 95% CI 1.7-18.2, p=0.005), left ventricular ejection fraction below 40% (HR, 4.88, 95% CI 1.26-18.9, p=0.022), hypertension (HR, 4.79, 95% CI 1.06-21.7, p=0.042), abnormal pulmonary function test (HR, 3.27, 95% CI 1.07-10.0, p=0.038), delayed myocardial hypersignal enhancement on cardiac magnetic resonance (HR, 2.26, 95% CI 0.25-20.4, p=0.003), and older age (HR per 10 years 1.69, 95% CI 1.13-2.52, p=0.01). The 10-year relapse-free survival rate for cardiac relapses was 53% (95% CI, 44-63). Baseline factors that were independently associated with cardiac relapse were kidney involvement (HR, 3.35, 95% CI 1.39-8.07, p=0.007), wall motion abnormalities (HR, 2.30, 95% CI 1.22-4.32, p=0.010), and left heart failure (HR 2.23, 95% CI 1.12-4.45, p=0.023). After adjustment for cardiac involvement severity, treatment with intravenous cyclophosphamide was associated with a lower risk of cardiac relapse (HR 0.16, 95% CI 0.033-0.78, p=0.024).

Conclusion:

• Our study identifies putative factors affecting morbidity and mortality in cardiac sarcoidosis patients. Intravenous cyclophosphamide is associated with lower relapse rates.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prognostic-factors-and-long-term-outcomes-in-cardiac-sarcoidosis/