Cardiac sarcoidosis treated with nonsteroidal immunosuppressive therapy

Kenichiro Suwa; Yoshihisa Naruse; Takeru Nabeta; Takeshi Kitai; Tatsunori Taniguchi; Kenji Yoshioka; Hidekazu Tanaka; Takahiro Okumura; Yuichi Baba; Yuya Matsue; Yuichiro Maekawa

doi:10.1016/j.ijcha.2024.101473

Cardiac sarcoidosis treated with nonsteroidal immunosuppressive therapy

Int J Cardiol Heart Vasc. 2024 Jul 23:53:101473. doi: 10.1016/j.ijcha.2024.101473. eCollection 2024 Aug.

Authors

Affiliations

¹ Division of Cardiology, Internal Medicine III, Hamamatsu University School of Medicine, Hamamatsu, Japan.
² Department of Cardiovascular Medicine, Kitasato University School of Medicine, Sagamihara, Japan.
³ Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan.
⁴ Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Osaka, Japan.
⁵ Department of Cardiology, Kameda Medical Center, Chiba, Japan.
⁶ Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Japan.
⁷ Department of Cardiology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
⁸ Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Nankoku, Japan.
⁹ Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Tokyo, Japan.

Abstract

Background: Nonsteroidal immunosuppressive therapy is a potential therapeutic strategy for cardiac sarcoidosis. However, it is not recommended as an established treatment option. This study aimed to demonstrate the clinical outcomes of patients with cardiac sarcoidosis using nonsteroidal immunosuppressants through the ILLUstration of the Management and PrognosIs of JapaNese PATiEnts with Cardiac Sarcoidosis multicenter retrospective registry.

Methods: From a cohort of 512 patients, 426 who received corticosteroid therapy and 26 who received other immunosuppressive therapy were included for analysis. Clinical outcomes included all-cause death, fatal ventricular arrhythmic events (FVAE), and worsening heart failure with hospitalization.

Results: Nonsteroidal immunosuppressants were used for retained fluorodeoxyglucose uptake in the heart (n = 14), corticosteroid side effects (n = 7), ventricular arrhythmia (n = 4), complete atrioventricular block (n = 2), worsened extracardiac sarcoidosis (n = 2), and other reasons (n = 2). They comprised of methotrexate (n = 20), cyclosporine (n = 2), cyclophosphamide (n = 2), and azathioprine (n = 3). After the addition of a nonsteroidal immunosuppressant, corticosteroids were reduced in 14 of 26 patients (5 [5-17] mg), although no patient discontinued corticosteroids. Of the 14 patients, decreased fluorodeoxyglucose uptake was observed in seven at follow-up. Clinical outcomes were observed in 11 patients (42.3 %). Detected events included all-cause death in five patients (19.2 %), FVAE in four (15.4 %), and worsening heart failure with hospitalization in five (19.2 %), with some overlap.

Conclusions: Nonsteroidal immunosuppressive therapy may be a possible treatment option for patients who are not stabilized with corticosteroids alone or develop corticosteroid side effects.

Keywords: Cardiac magnetic resonance; Cardiac sarcoidosis; Fluorine-18 fluorodeoxyglucose positron emission tomography; Late gadolinium enhancement; Nonsteroidal immunosuppressive therapy.