Cardiac Sarcoidosis treated with non-steroidal immunosuppressive therapy

Abstract

Abstract Background Non-steroidal immunosuppressive therapy is a potential treatment strategy for cardiac sarcoidosis. However, due to the lack of robust evidence, it is not recommended as an established treatment option in the guideline. Purpose The purpose of the study is to demonstrate the clinical outcome of the patients with cardiac sarcoidosis using non-steroidal immunosuppressive therapy in a retrospective registry entitled ILLUMINATE-CS (ILLUstration of the Management and prognosIs of JapaNese pATiEnts with Cardiac Sarcoidosis). Methods Of an entire cohort of 512 patients, 26 who received immunosuppressive therapy other than steroids were recruited for the analysis. The clinical outcome included all-cause death, fatal ventricular arrhythmic event (FVAE), and worsening heart failure with hospitalization. Results The reasons to use non-steroidal immunosuppressant were increased fluorodeoxyglucose (FDG) accumulation on the heart suggesting worsened inflammation (n = 13), side effects of steroid (n = 7), ventricular tachycardia or frequent ventricular premature contraction (n=4), complete atrioventricular block (n = 2), worsened symptom relating to non-cardiac sarcoidosis including neuro and pulmonary sarcoidosis (n = 2), and the other reason (n = 2), with some overlapping. Used non-steroidal immunosuppressants were comprised of methotrexate (n = 20, 6.9 ± 2.1 mg/week), cyclosporine (n =2, 75 or 100 mg/day), cyclophosphamide (n = 2, 25 or 50 mg/day), and azathioprine (n = 3, 100 mg/day in each). Side effects of non-steroidal immunosuppressant were observed in six cases and included one with methotrexate-related interstitial pneumonia, one with a methotrexate-associated lymphoproliferative disorder, one with septic coxitis with methotrexate, one with methotrexate-induced acute kidney injury, one with methotrexate-induced hepatic dysfunction, and one with azathioprine-induced anemia. After the addition of a non-steroidal immunosuppressant, steroids could be reduced in 14 cases of 26 cases with a median maintenance dose of 5 (interquartile range of 5–17) mg, although no cases stopped steroids. Of 13 cases who started non-steroidal immunosuppressant for the increased FDG uptake, decreased FDG uptake was observed in seven cases at the follow-up scan. Clinical outcome events were observed in 11 cases (42.3%). Detected events included all-cause death in five cases (19.2%), FVAE in four cases (15.4%), and worsening heart failure with hospitalization in five cases (19.2%), with some overlapping. Conclusions Non-steroidal immunosuppressive therapy may be a possible treatment option for patients who are not stabilized using sole steroid therapy or who suffer from the side effects of steroids.