Cardiac sarcoidosis (CS) is a life-threatening condition in which clear recommendations are lacking. We aimed to systematically review the literature on cardiac sarcoidosis treated by corticosteroids and/or immunosuppressive agents in order to update the management of CS.

Using PubMed, Embase and Cochrane Library databases, we found original articles on corticosteroid and standard immunosuppressive therapies for CS that provided at least a fair Scottish Intercollegiate Guidelines Network (SIGN) overall assessment of quality and we analysed the relapse rate, major cardiac adverse events (MACEs) and adverse events. We based our methods on the PRISMA statement and checklist.

We retrieved 21 studies. Mean quality provided by SIGN assessment was 6.8 out of 14 (range 5–9). Corticosteroids appeared to have a positive impact on left ventricular function, atrioventricular block and ventricular arrhythmias. For corticosteroids alone, nine studies (45%, n=351) provided data on relapses, representing an incidence of 34% (n=119). Three studies (14%, n=73) provided data on MACEs (n=33), representing 45% of MACEs in patients treated by corticosteroid alone. Nine studies provided data on adjunctive immunosuppressive therapy, of which four studies (n=78) provided data on CS relapse, representing an incidence of 33% (n=26). Limitations consisted of no randomised control trial retrieved and unclear data on MACEs in patients treated by combined immunosuppressive agents and corticosteroids.

Corticosteroids should be started early after diagnosis but the exact scheme is still unclear. Studies concerning adjunctive conventional immunosuppressive therapies are lacking and benefits of adjunctive immunosuppressive therapies are unclear. Homogenous data on CS long-term outcomes under corticosteroids, immunosuppressive therapies and other adjunctive therapies are lacking.

心脏结节病 (CS) 是一种危及生命的疾病，缺乏明确的建议。我们旨在系统地回顾关于用皮质类固醇和/或免疫抑制剂治疗心脏结节病的文献，以更新 CS 的管理。

使用 PubMed、Embase 和 Cochrane Library 数据库，我们找到了关于皮质类固醇和标准免疫抑制疗法的原始文章，这些文章至少提供了公平的苏格兰校际指南网络 (SIGN) 对质量的总体评估，我们分析了复发率、主要心脏不良事件。 MACE) 和不良事件。我们的方法基于 PRISMA 声明和清单。

我们检索了 21 项研究。SIGN 评估提供的平均质量为 6.8 / 14（范围 5-9）。皮质类固醇似乎对左心室功能、房室传导阻滞和室性心律失常有积极影响。仅对于皮质类固醇，9 项研究（45%，n=351）提供了复发数据，代表 34%（n=119）的发生率。三项研究（14%，n=73）提供了有关 MACE 的数据（n=33），占仅接受皮质类固醇治疗的患者中 MACE 的 45%。九项研究提供了辅助免疫抑制治疗的数据，其中四项研究（n=78）提供了 CS 复发的数据，发生率为 33%（n=26）。局限性包括没有检索到随机对照试验，以及接受联合免疫抑制剂和皮质类固醇治疗的患者的 MACE 数据不明确。

皮质类固醇应在诊断后及早开始，但确切的方案仍不清楚。缺乏关于辅助常规免疫抑制疗法的研究，辅助免疫抑制疗法的益处尚不清楚。缺乏关于皮质类固醇、免疫抑制疗法和其他辅助疗法下 CS 长期结果的同质数据。