The Many Faces of Cardiac Sarcoidosis

Virian D. Serei, MD, PhD; Billie Fyfe, MD


Am J Clin Pathol. 2020;153(3):294-302. 

In This Article

Current Treatment

Although specific guidelines do not exist, treatment of cardiac sarcoidosis involves immunosuppression and management of cardiac dysfunction.[4,28] Corticosteroids are the first-line immunosuppressive treatment for cardiac sarcoidosis.[4,28] As an adjunct to corticosteroid therapy, steroid-sparing agents such as hydroxychloroquine, azathioprine, and methotrexate may be used and have been shown to be beneficial.[4,28] Although immunosuppression can eliminate the presence of cardiac sarcoid granulomas, fibrosis is irreversible and may permanently impair cardiac function.[16] Arrhythmias are managed with antiarrhythmics and/or with placement of an automatic implantable cardioverter-defibrillator.[4,29] Ventricular dysfunction leading to heart failure is managed according to heart failure management guidelines.[4,28]

Cardiac transplantation is reserved for patients in severe end-stage heart failure refractory to medical management.[4] Mechanical circulatory support such as a left ventricular assist device is often used as bridge to transplantation.[30] Other uses for mechanical circulatory support in heart failure patients include long-term destination therapy and bridge to recovery. However, to our knowledge, there is no published literature describing the use of mechanical circulatory support as destination therapy or bridge to recovery for cardiac sarcoid patients. It is likely that dense fibrosis, which is characteristic of end-stage disease, significantly and permanently impairs cardiac function, necessitating cardiac transplantation and precluding bridge to recovery (Image 3).