Corticosteroid treatment in patients with newly diagnosed clinically silent cardiac sarcoidosis is associated with significantly improved long-term outcomes

Background: In patients with sarcoidosis, cardiovascular multimodality imaging techniques have revealed that cardiac involvement is detected four to five times more frequently than what is clinically apparent. Despite the increasing prevalence of patients with cardiac sarcoidosis (CS) diagnosed at a subclinical stage, their long-term outcomes are not well known and current guidelines are inconsistent regarding the management of silent CS, including the use of corticosteroids after diagnosis.

Objectives: To investigate the incidence of adverse long-term outcomes and the predictive value of corticosteriods in newly diagnosed patients with clinically silent CS.

Methods: This retrospective study comprised 43 patients with newly diagnosed clinically silent CS, with available follow-up and cardiovascular magnetic resonance (CMR) assessment at baseline. Silent CS was defined as CS in patients with biopsy-proven extracardiac sarcoidosis without cardiac symptoms but with abnormalities on CMR or positron emission tomography according to Heart Rhythm Society criteria. The primary endpoint was a composite of all-cause mortality, new ventricular arrhythmia or atrioventricular block requiring cardiac device implantation, and hospitalization for heart failure.

Results: At baseline, patients presented a significant extent of left ventricular (LV) late gadololinium extent (12±4 % of LV Mass), despite having normal LV ejection fraction (58±4 %). 58.1% of the patients received immunosuppressive therapy with corticosteroids after CS diagnosis. Over a follow-up period of 5 years,  44.2% of patients reached the composite endpoint. Patients treated with corticosteroids after CS diagnosis had a significantly lower incidence of adverse outcomes during follow-up compared with patients not treated with corticosteroids (28% vs 66.7 %, Log Rank P=0.008). In multivariable regression analysis, treatement with corticosteroids was significantly independently associated with improved outcomes (HR 7.69, 95% CI 1.11-11.11, P=0.044).

Conclusion:

Patients with clinically silent CS treated with corticosteroids after diagnosis have significantly better long-term outcomes. The potential of corticosteroids to improve long-term outcomes in asymptomatic CS should be investigated in larger studies.