Cardiac sarcoidosis: A long term follow up study.

Patrice Cacoub,David Saadoun,Matthieu Resche-Rigon,Anne Claire Desbois,Lucie Biard,Catherine Chapelon-Abric,Claudio Passino

doi:10.1371/journal.pone.0238391

Abstract

BackgroundPrognostic factors are lacking in cardiac sarcoidosis (CS), and the effects of immunosuppressive treatments are unclear.ObjectivesTo identify prognostic factors and to assess the effects of immunosuppressive drugs on relapse risk in patients presenting with CS.MethodsFrom 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.ResultsThe 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), areas of late gadolinium 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).ConclusionsOur study identifies putative factors affecting morbidity and mortality in cardiac sarcoidosis patients. Intravenous cyclophosphamide is associated with lower relapse rates.

Highlights

Sarcoidosis is a multi-system granulomatous disease of unknown origin with an overall prevalence from 10 to 20 per 100,000 in white American and European patients to 35 per 100,000 in African American patients [1,2,3]
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), areas of late gadolinium 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)
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)

Summary

Introduction

Sarcoidosis is a multi-system granulomatous disease of unknown origin with an overall prevalence from 10 to 20 per 100,000 in white American and European patients to 35 per 100,000 in African American patients [1,2,3]. Manifest cardiac involvement—known as cardiac sarcoidosis (CS)—occurs in 5% to 11% [4,5,6] whereas cardiac involvement was found in 25% of patients with sarcoidosis on autopsies [7, 8]. Such findings are consistent with data using late gadolinium enhancement on cardiac magnetic resonance imaging (MRI) [9, 10]. Between 16% and 35% of patients presenting with complete atrioventricular block [11, 12] or ventricular tachycardia of unknown etiology [12,13,14] have previously undiagnosed CS. Editor: Claudio Passino, Ospedale del Cuore G Pasquinucci Fondazione Toscana Gabriele Monasterio di Massa, ITALY

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