Cardiac sarcoidosis: Diagnosis, therapeutic management and prognostic factors

Abstract

Cardiac sarcoidosis (CS) is a severe localization, observed mostly in cardiology departments. Despite appropriate cardiological treatment, CS is a potentially life-threatening condition, and accounts for 13-85% of sarcoidosis-related deaths. This retrospective study aimed to give an overview of CS in a non-cardiac unit, to analyse the effect of first-line immunosuppressive treatment on outcome and survival and to evaluate factors associated with relapses. From 534 cases of sarcoidosis, we selected 59 patients with CS according to "2006 international criteria". We performed an in-depth analysis regarding symptoms, physical signs and cardiac investigation results. Patients were followed for a median period of 60 months. The median age at cardiac signs was 42 years. Echocardiography abnormalities, isotopic defects and abnormal magnetic resonance imaging findings were observed in 81%, 84% and 92% of patients, respectively. First-line treatment included steroids alone in 24 patients and steroids plus immunosuppressive therapy in 35 patients. Forty-seven (80%) patients recovered; 12 stabilized or worsened. The recovery rate was 75% in the steroids alone group versus 83% in the steroids plus immunosuppressive therapy group. Five (9%) patients died during follow-up, with two deaths attributed to CS. The overall 1- and 5-year survival rates were 98% and 92%, respectively. Our series of patients with CS who received steroids alone or combined with immunosuppressive therapy had a good prognosis, with an overall 5-year survival rate of 92%. The recovery rate was 85%, with no significant difference between patients treated with steroids alone or plus immunosuppressive therapy.