Clinical and imaging response to tumor necrosis factor alpha inhibitors in treatment of cardiac sarcoidosis: a multicenter experience.

Abstract Background Cardiac sarcoidosis (CS) is an increasingly recognized cause of cardiomyopathy, however data on immunosuppressive strategies are limited. Treatment with tumor necrosis factor (TNF) alpha inhibitors is not well described; moreover, there may be heart failure (HF)-related safety concerns. Methods Retrospective multicenter study of TNF alpha inhibitor treated CS patients. Baseline characteristics, treatments, and outcomes were adjudicated. Results Thirty-eight patients with CS (mean 49.9 years old, 42% women, 53% African American) were treated with TNF alpha inhibitor (30 infliximab, 8 adalimumab). Prednisone dose decreased from time of TNF alpha inhibitor initiation (21.7±17.5 mg) to 6-months (10.4±6.1 mg, p=0.001) and 12-months post (7.3±7.3 mg, p=0.002). On pre-TNF alpha inhibitor treatment 18-flourodoxyglucose position emission tomography (FDG-PET), 84% of patients had cardiac FDG uptake. Post-treatment, there was a significant decrease in number of segments involved (3.5±3.8 to 1±2.5, p=0.008) and maximum standardized uptake value (3.59±3.7 to 0.57±1.6, p=0.0005), with 73% of patients demonstrating complete resolution or improvement of cardiac FDG uptake. Left ventricular ejection fraction remained stable (45±16.5 to 47±15.0%, p=0.10). Four patients required inpatient HF treatment, and 8 had infections; 2 required treatment cessation. Conclusions TNF alpha inhibitor treatment guided by FDG-PET may minimize corticosteroid use and effectively reduce cardiac inflammation without significant adverse effect on cardiac function. However infections were common, some of which were serious, and therefore patients require close monitoring for both infection and cardiac symptoms.