Cardiac sarcoidosis: Diagnosis, therapeutic management and prognostic factors

Arch Cardiovasc Dis. 2017 Aug-Sep;110(8-9):456-465. doi: 10.1016/j.acvd.2016.12.014. Epub 2017 May 26.


Background: 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.

Aims: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords: Cardiac sarcoidosis; Corticoïdes; Cyclophosphamide; Methotrexate; Méthotrexate; Rechute taux de guérison; Relapse; Sarcoïdose cardiaque; Steroids; Survival rate.