Review Article: PDF OnlySarcoid Heart Disease Review of Current KnowledgeJin, Ch Chengyue MD1; Gandrabur, Liliya MD2; Kim, Woo Young MD1; Pan, Stephen MD,MS3; Ash, Julia Y. MD.2Author Information 1Department of Medicine, Westchester Medical Center, Valhalla, NY 2Department of Medicine, Division of Rheumatology, Westchester Medical Center, Valhalla, NY 3Department of Medicine and Cardiology, Westchester Medical Center, Valhalla, NY Funding: None. Conflict of Interest: None. Corresponding Author: Julia Y. Ash, MD Division of Rheumatology Westchester Medical Center 100 Woods Road, Valhalla, NY 10595 [email protected] 914-493-7360 Cardiology in Review: June 14, 2021 - Volume - Issue - doi: 10.1097/CRD.0000000000000400 Buy PAP Metrics Abstract Sarcoidosis is a granulomatous disease with the potential of multiple organ system involvement and its etiology remains unknown. Cardiac involvement is associated with worse clinical outcome, and has been reported to be 20%-30% in Caucasian and as high as 58% in Japanese populations with sarcoidosis. Clinical manifestations of cardiac sarcoidosis highly depend on the extent and location of granulomatous inflammation. The most frequent presentations include heart block, tachyarrhythmia, or heart failure. Endomyocardial biopsy is the most specific diagnostic test, but has poor sensitivity due to often patchy involvement. The diagnosis of cardiac sarcoidosis remains challenging due to non-specific imaging findings. Both 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) and cardiac magnetic resonance imaging can be used to evaluate cardiac sarcoidosis, but evaluate different stages of the disease process. FDG-PET detects metabolically-active inflammatory cells while cardiac magnetic resonance imaging with late gadolinium enhancement reveals areas of myocardial necrosis and fibrosis. Aggressive therapy of symptomatic cardiac sarcoidosis is often sought due to the high risk of sudden death and/or progression to heart failure. Prednisone 20-40 mg a day is the recommended initial treatment. In refractory or severe cases, higher doses of prednisone, 1 to 1.5 mg/kg/day (or its equivalent) and addition of a steroid sparing agent have been utilized. Methotrexate is added most commonly. Long-term improvement has been reported with the use of a combination of weekly methotrexate and prednisone versus prednisone alone. After initiation of treatment, a cardiac FDG-PET scan may be performed 2-3 months later to assess treatment response. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.