Long-Term Corticosteroid-Sparing Immunosuppression for Cardiac Sarcoidosis. J Am Heart Assoc 2019 Sep 17;8(18):e010952
Date
09/21/2019Pubmed ID
31538835Pubmed Central ID
PMC6818011DOI
10.1161/JAHA.118.010952Scopus ID
2-s2.0-85072393038 (requires institutional sign-in at Scopus site) 58 CitationsAbstract
Background Long-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 Results Retrospective 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. Conclusions Corticosteroid-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.
Author List
Rosenthal DG, Parwani P, Murray TO, Petek BJ, Benn BS, De Marco T, Gerstenfeld EP, Janmohamed M, Klein L, Lee BK, Moss JD, Scheinman MM, Hsia HH, Selby V, Koth LL, Pampaloni MH, Zikherman J, Vedantham VMESH terms used to index this publication - Major topics in bold
AdalimumabAnti-Inflammatory Agents
Arrhythmias, Cardiac
Cardiomyopathies
Deprescriptions
Drug Therapy, Combination
Electrocardiography
Female
Fluorodeoxyglucose F18
Glucocorticoids
Humans
Immunosuppressive Agents
Maintenance Chemotherapy
Male
Methotrexate
Middle Aged
Positron-Emission Tomography
Prednisone
Radiopharmaceuticals
Recurrence
Retrospective Studies
Sarcoidosis
Treatment Outcome