Background: Corticosteroids are first line agents for the treatment of active Cardiac Sarcoidosis (CS). Conflicting data exists for improvement of CS symptoms and outcomes with corticosteroids, while side effects of chronic steroid use have been well studied and known to be detrimental. Second line medications commonly used include methotrexate and TNF-alpha. Cardiac FDG-PET scans have utility in monitoring disease activity in response to these medications.
Objective: To determine the treatment success of corticosteroid monotherapy, as defined by resolution of PET activity, and quantify the utility of PET scans in the management of patients with active CS.
Methods: 538 patients with suspected CS were referred to a specialized CS clinic for evaluation for the presence of CS. Patient demographics, symptoms and investigations were collected retrospectively. Patients diagnosed with CS were started on prednisone, slowly weaned off, and completed post-treatment PET scans. The results of post-treatment PET scans helped guide dosing and addition of second line agents.
Results: Of the 538 patients assessed, 97 were diagnosed with CS using endomyocardial biopsy or advanced cardiac imaging. Of these, 89 were treated with prednisone. Once their dose was weaned down to a maintenance dose of 20mg/day, 64 patients completed post-treatment PET scans. Of these, 30 were negative for CS pattern uptake - an improvement in 46.9% of patients with prednisone. The remaining 34 (53.1%) had persistent positive cardiac uptake. Of the patients with continued active inflammation, dosing of prednisone was either increased and/or methotrexate was added. 19 of the 34 who had a positive post treatment PET scan began a course of methotrexate, as well as completed a second post treatment PET scan, where 11 continued to show active, patchy inflammation.
Conclusion: Corticosteroid monotherapy resulted in suboptimal suppression of inflammation in the majority of patients with active CS. Cardiac uptake on FDG-PET scans in CS patients, as a measure of prednisone efficacy, is useful in guiding dosing and/or the need for additional medication therapy for persistent CS such as methotrexate and TNF-alpha. Early use of second-line agents such as methotrexate should be studied in this population.