Long‐Term Corticosteroid‐Sparing Immunosuppression for Cardiac Sarcoidosis

Abstract

BackgroundLong‐term corticosteroid therapy is the standard of care for treatment of cardiac sarcoidosis (CS). The efficacy of long‐term corticosteroid‐sparing immunosuppression in CS is unknown. The goal of this study was to assess the efficacy of methotrexate with or without adalimumab for long‐term disease suppression in CS, and to assess recurrence and adverse event rates after immunosuppression discontinuation.Methods and ResultsRetrospective chart review identified treatment‐naive CS patients at a single academic medical center who received corticosteroid‐sparing maintenance therapy. Demographics, cardiac uptake of 18‐fluorodeoxyglucose, and adverse cardiac events were compared before and during treatment and between those with persistent or interrupted immunosuppression. Twenty‐eight CS patients were followed for a mean 4.1 (SD 1.5) years. Twenty‐five patients received 4 to 8 weeks of high‐dose prednisone (>30 mg/day), followed by taper and maintenance therapy with methotrexate±low‐dose prednisone (low‐dose prednisone, <10 mg/day). Adalimumab was added in 19 patients with persistently active CS or in those with intolerance to methotrexate. Methotrexate±low‐dose prednisone resulted in initial reduction (88%) or elimination (60%) of 18‐fluorodeoxyglucose uptake, and patients receiving adalimumab‐containing regimens experienced improved (84%) or resolved (63%) 18‐fluorodeoxyglucose uptake. Radiologic relapse occurred in 8 of 9 patients after immunosuppression cessation, 4 patients on methotrexate‐containing regimens, and in no patients on adalimumab‐containing regimens.ConclusionsCorticosteroid‐sparing regimens containing methotrexate with or without adalimumab is an effective maintenance therapy in patients after an initial response is confirmed. Disease recurrence in patients on and off immunosuppression support need for ongoing radiologic surveillance regardless of immunosuppression regimen.

Heart Failure Association of the ESC, Heart Failure Society of America and Japanese Heart Failure Society Position statement on endomyocardial biopsy

“…101 Based on small observational cohorts, clinical experience and expert opinion, immunosuppressive therapy can be instituted in virus-negative eosinophilic myocarditis, ICI-mediated myocarditis, cardiac sarcoidosis and myocarditis associated with autoimmune diseases. 17,52,[102][103][104] In patients with myocarditis of unknown aetiology, a clinical trial failed to demonstrate a beneficial effect of immunosuppression on LV function and survival. 105 In patients with DCM, therapeutic implications of EMB-proven virus-negative myocardial inflammation (i.e.…”

Biologic Therapies in Sarcoidosis and Uveitis: A Review

“…Thirty articles included for the final review are composed of two systematic reviews with metaanalysis [11,12], fifteen case reports/series [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27], and thirteen observational studies [1,3,4,9,[28][29][30][31][32][33][34][35][36][37]. The two systematic reviews provided data on 692 patients.…”

“…There is literature describing the use of adalimumab in extra-pulmonary sarcoidosis refractory to steroids. These include studies by Rosenthal et al, a retrospective study involving 28 patients with cardiac sarcoidosis wherein adalimumab was successfully used in combination with methotrexate to achieve notable suppression in myocardial inflammation [ 32 ]. The study recommends a strategy of long term combination therapy of methotrexate and adalimumab to achieve disease quiescence in cardiac sarcoidosis.…”

Cardiac sarcoidosis: diagnosis and management

“…The scan continues to show FDG uptake in mediastinal lymph nodes (Bremer et al, 2018) . who discontinue immunosuppression have increased risk of radiologic recurrence and recurrent VT (Rosenthal et al, 2019). Furthermore, immunosuppression with prednisone and methotrexate showed lower risk for recurrence, lower steroid dose and less side effects compared to treatment solely with prednisone (Ballul et al, 2019).…”