TY - JOUR T1 -心源性结节病:JF -欧洲呼吸杂志JO - Eur Respir J DO - 10.1183/13993003.00449-2021 SP - 2100449 AU - Stievenart, Julien AU - Le Guenno, Guillaume AU - Ruivard, Marc AU - Rieu, Virginie AU - André， Marc AU - Grobost，背景心性结节病(CS)是一种危及生命的疾病，缺乏明确的治疗建议。我们的目的是系统地回顾糖皮质激素和/或免疫抑制剂治疗心脏结节病的文献，以更新CS的管理。方法利用Pubmed、Embase和Cochrane Library数据库，查找有关皮质类固醇和/或标准免疫抑制治疗CS的原始文章，至少提供公平的SIGN总体质量评估，并分析复发率、主要心脏不良事件(mace)和不良事件。我们的方法基于Prisma语句和清单。结果检索到21篇研究。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室功能、房室传导阻滞和室性心律失常有积极影响。仅就皮质类固醇而言，9项(45%)研究(n=351)提供了复发的数据，发生率为34% (n=119)。三项研究(14%，n=73)提供了mace (n=33)的数据，代表了仅接受皮质类固醇治疗的患者中45%的mace。 Nine studies provided data on adjunctive immunosuppressive therapy in which four studies (n=78) provided data on CS relapse, representing an incidence of 33% (n=26). Limitations consisted in no randomised control trial retrieved and unclear data on MACEs in patients treated by combined immunosuppressive agents and corticosteroids.Conclusions 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.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. STIEVENART has nothing to disclose.Conflict of interest: Dr. Le Guenno has nothing to disclose.Conflict of interest: Dr. RUIVARD has nothing to disclose.Conflict of interest: Dr. RIEU has nothing to disclose.Conflict of interest: Dr. ANDRE has nothing to disclose.Conflict of interest: Dr. vincent has nothing to disclose. ER -