Department of Medicine-DIMED, Unit of Rheumatology, Padova University Hospital, Padova, Italy.
Department of Rheumatology, Faculty of Medicine, University of Debrecen, Hungary.
Autoimmun Rev. 2024 Jan;23(1):103463. doi: 10.1016/j.autrev.2023.103463. Epub 2023 Oct 14.
Systemic sclerosis (SSc) is a heterogeneous autoimmune disease, where a significant proportion of patients develop interstitial lung disease (ILD), which is the major cause of mortality. In recent years, the diagnosis of SSc-ILD has improved a lot, and caring rheumatologists, together with pulmonologists, regularly screen and follow the development and course of ILD. Considerable progress has also been made in the treatment of SSc-ILD based on several clinical trials. The recommendations for immunosuppressive treatment have been modified and supplemented with targeted agents (tocilizumab, rituximab), and antifibrotic drugs such as nintedanib registered as a new treatment for SSc-ILD. However, there are no clear recommendations regarding the start and timing of nintedanib treatment. A debate on the early introduction of nintenadib or not took place on the 7th edition of the International Congress on Controversies in Rheumatology and Autoimmunity (CORA) in March/2023, and this review summarizes the main arguments that were discussed in this session.
系统性硬化症（SSc）是一种异质性自身免疫性疾病，其中相当一部分患者会出现间质性肺疾病（ILD），这是导致死亡的主要原因。近年来，SSc-ILD 的诊断有了很大的提高，风湿病学家与肺科医生一起定期对ILD 的发展和病程进行筛查和随访。基于几项临床试验，SSc-ILD 的治疗也取得了相当大的进展。免疫抑制治疗的建议已经进行了修改和补充，包括靶向药物（托珠单抗、利妥昔单抗）和已注册为 SSc-ILD 新疗法的抗纤维化药物尼达尼布。然而，对于尼达尼布治疗的开始和时机，尚无明确的建议。在 2023 年 3 月举行的第 7 届国际风湿病与自身免疫学争议大会（CORA）上，就是否应尽早引入尼达尼布进行了一场辩论，本文总结了本次会议讨论的主要观点。
