Combination Therapy with Prednisone and Mycophenolate Mofetil in the Management of Cardiac Sarcoidosis

Introduction Cardiac sarcoidosis (CS) is characterized histologically by the presence of noncaseating granulomas and manifests as conduction abnormality, ventricular arrhythmia and progressive heart failure. With advances in imaging techniques, CS is being increasingly recognized as an underlying cause of cardiomyopathy. To date, there are no randomized trials or published guidelines to direct treatment strategies in CS, specifically in regards to optimal immunosuppressive therapy. We sought to describe our experience using a combination of prednisone and mycophenolate mofetil (MMF) in the treatment of patients (pts) with CS. Methods We retrospectively identified pts with CS who received either prednisone monotherapy or combination therapy with MMF. Outcomes assessed were response to immunosuppression based on occurrence of post-treatment ventricular arrhythmias, change in left ventricular ejection fraction (LVEF) on echocardiogram ( recovery defined as LVEF ≥ 50%, improvement defined as any increase in LVEF) and change in inflammation on 18 F-fluorodeoxyglucose positron emission tomography ( 18 F-FDG PET). Results Thirty-seven pts with CS were identified, 9 of whom were classified as having isolated CS. A total of 25 pts (51.5 ± 11.4 yrs old, 40% female, 40% African American (AA)) were treated with combination therapy: 16 were initiated on MMF within 60 days of prednisone and 9 greater than 60 days after prednisone. Twelve pts (57.2 ± 8.8 yrs old, 50% female, 42% AA) were treated with prednisone only. The combination therapy group, when compared to the prednisone only group, were sicker at presentation and more commonly presented with ventricular tachycardia (VT) (8/25, 32% vs 1/12, 8% respectively), advanced AV block (7/25, 28% vs 2/12, 16.7%) and EF 18 F-FDG PET of which 6 (31.6%) showed complete resolution of inflammation and 8 (42.1%) showed improvement. MMF was generally well tolerated in this cohort. Conclusion We found that patients with severe CS appear to respond well to combination therapy with prednisone and MMF, with more than half having improvement in LV function and two-thirds showing improvement in FDG uptake. Those with severe LV dysfunction at treatment initiation however, are less likely to recover LV function. Our experience suggests that MMF is an effective steroid-sparing therapy in patients with CS.