Treatment of Active Cardiac Sarcoidosis with TNF Alpha Inhibitors

Abstract

Background Cardiac sarcoidosis (CS) is associated with significant morbidity and mortality including progressive heart failure and sudden cardiac death. High-dose steroid immunosuppression remains standard of care for CS but there is a lack of randomized clinical trial data. As a result, there remains significant treatment variability among specialized centers. TNF alpha inhibitors are widely used for other inflammatory diseases and are generally well tolerated. However, little data, if any, exists regarding their utility in CS. TNF alpha inhibitors are particularly attractive given the potential adverse effects of chronic glucocorticoid based immunosuppression. The purpose of this study was to explore the effectiveness of TNF alpha inhibitors in active CS. Methods We performed a retrospective analysis of patients with metabolically active CS (defined by increased FDG avidity by cardiac PET imaging) who were treated with TNF alpha inhibitors. All patients underwent follow up PET imaging. Disease activity was quantified by the number of left ventricular (LV) myocardial segments with FDG avidity (based on a 17 segment model of the LV). Adverse events related to therapy was also recorded. Results Nine patients treated with TNF alpha inhibitors were included in this analysis. Eight Patients received the TNF alpha inhibitor, infliximab, one patient received adalimumab. Eight of the nine patients had previously received prednisone for CS; four transitioned from prednisone to infliximab due to adverse effects of steroid use; three transitioned because of continued active CS despite steroid therapy; one patient exhibited recurrent cardiac FDG avidity after prednisone discontinuation and another declined steroid treatment. The number of LV myocardial segments with active CS decreased from 8.6 +/- 3.6 to 1.9 +/- 3.5 (p=0.001) after a mean treatment duration of 7 months of infliximab that represents a decline of FDG avid LV segments by 72%. There was no significant change in LV ejection fraction (45 +/- 15% to 50 +/- 11% after therapy, p=NS). Six of the nine patients had complete resolution of active CS, and two had partial improvement. One patient experienced pancytopenia felt possibly related to infliximab; no other obvious adverse events were observed. Conclusions In this retrospective cohort, TNF alpha inhibition lead to significant improvement in disease activity based on FDG PET imaging. Most patients demonstrated complete resolution of active CS with minimal adverse effects. Further investigation into the use of TNF alpha inhibitors for treating active CS is warranted.