TY - JOUR T1 -心脏结节病系统回顾文献对皮质类固醇和免疫抑制疗法JF -欧洲呼吸杂志》乔和J - 10.1183/13993003.00449 -2021欧元SP - 2100449 AU Stievenart朱利安AU - Le Guenno Guillaume盟——Ruivard Marc盟——Rieu小薇吉妮盟——安德烈,马克•AU - Grobost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有关皮质类固醇和/或免疫抑制剂治疗心脏结节病的文献，以更新CS的管理。方法利用Pubmed、Embase和Cochrane Library数据库，我们找到了关于皮质类固醇和/或标准免疫抑制治疗CS的原始文献，这些文献至少提供了公平的SIGN总体质量评估，并分析了复发率、主要心脏不良事件(MACEs)和不良事件。我们的方法基于Prisma语句和检查表。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室功能、房室传导阻滞和室性心律失常有积极影响。仅皮质类固醇，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的数据(n=33)，占单独使用皮质类固醇治疗的患者mace的45%。 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 -