On Managing Cardiac Sarcoidosis

By E. Ashley Hardin, M.D., Assistant Professor of Internal Medicine, and Vlad G. Zaha, M.D., Ph.D., Assistant Professor of Internal Medicine

At #AHA20 this year, we attended Dr. Neal Lakdawala’s lecture “Cardiac Sarcoid: When and How to Treat Inflammation.” He provided updates on the treatment of cardiac sarcoidosis (CS), a diagnosis that is increasingly recognized. Several key points from his lecture are worth noting. Sarcoidosis is an idiopathic, multisystem disorder that involves the heart in about 25% of cases. The hallmark histopathologic diagnosis is that of non-caseating granulomas without an alternative cause. The diagnosis of CS presents some of the major diagnostic and therapeutic challenges and can be associated with significant morbidity and mortality. CS often manifests as heart block, ventricular arrhythmias, or heart failure.

As reflected in current guidelines, treatment of CS with immunosuppression relies heavily on a tissue diagnosis (either cardiac or extra-cardiac) and depends on the presence of arrhythmias or left ventricular systolic dysfunction along with evidence of cardiac inflammation on imaging (cardiac MRI and/or cardiac 18F-fluorodeoxyglucose positron emission tomography (FDG-PET)).

Management of CS is generally focused on treatment of inflammation and of its clinical sequelae (arrhythmias and ventricular dysfunction). Unfortunately, the data for management of CS are largely based on single-center experiences. The CHASM-CS-RCT is the first open-label, randomized trial for CS therapy, currently enrolling treatment-naïve patients who have active inflammation on cardiac FDG-PET. Experts tend to agree that first-line therapy consists of prednisone at 0.5mg/kg/day with short-term assessment of response (1-3 months) via repeat cardiac imaging. If needed, a variety of steroid-sparing agents have been used and include methotrexate (most commonly), mycophenolic acid, azathioprine, and several biologic agents, including infliximab and other TNF alpha antagonists.

The use of device therapy is recommended for patients with advanced heart block and malignant ventricular arrhythmias. Referral to a center with expertise is recommended. At UT Southwestern, we have a multidisciplinary approach to the management of these complex patients. We offer cardiac MRI with multiparametric myocardial tissue characterization protocols, as well as cardiac FDG-PET imaging, for diagnosis and longitudinal monitoring of myocardial inflammatory changes and their impact on the cardiac structure and function in patients with CS. Patients are monitored longitudinally in a dedicated CS clinic, and there is close collaboration with the electrophysiology service when device therapy is indicated.