Treatment of cardiac sarcoidosis.

Abstract

In sarcoidosis, the appropriate management strategy remains challenging especially because of the lack of confident diagnosis, considerable variability in initial presentation, disease evolution, and outcome. Although asymptomatic patients with limited cardiac involvement have been described to have a benign outcome, cardiac sarcoidosis is associated with high morbidity and mortality and even sudden cardiac death in a significant proportion of patients. Higher morbidity and mortality can be related with both the disease activity and extent of fibrosis. Historical series suggested a 5-year mortality rate of 60% in patients with cardiac sarcoidosis. This has definitely improved with the appropriate use of anti-inflammatory medications as well as heart failure treatment, antiarrhythmic medication and device implantation. Timely recognition and vigorous initial approach is essential in avoiding life-threatening arrhythmias and sudden cardiac death. Advanced imaging modalities have proven to be helpful in the diagnostic approach and guiding treatment decisions. However, there is no optimal screening and risk stratification strategy available and further studies are required to determine, which patients would benefit from the available treatments. This review concentrates on the broad principles of management in cardiac sarcoidosis and the efficacy of sarcoidosis-specific medication and cardiac-specific therapies for cardiac dysfunction and rhythm disturbances.