Toronto Scleroderma Program, Schroeder Arthritis Institute, Toronto Western Hospital, Mount Sinai Hospital, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
Columbia University Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, New York, USA.
Curr Opin Rheumatol. 2024 Nov 1;36(6):420-426. doi: 10.1097/BOR.0000000000001049. Epub 2024 Aug 27.
Interstitial lung disease (ILD) is the leading cause of death in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). The American College of Rheumatology (ACR), in conjunction with the American College of Chest Physicians (CHEST), recently published clinical practice guidelines for the treatment of adults with systemic autoimmune rheumatic disease-associated ILD, including SSc-ILD. Herein, we summarize evidence from randomized trials evaluating the safety and efficacy of pharmacologic therapies for the treatment of SSc-ILD.
In this review, we present findings from recent randomized controlled trials in SSc-ILD. The pharmacologic therapies discussed include immunosuppressive medications (mycophenolate, cyclophosphamide, rituximab, and tocilizumab) and antifibrotic medications (nintedanib and pirfenidone).
Randomized trials provide an evidence base for the SSc-ILD treatment recommendations put forth in the ACR/CHEST Guidelines for the treatment of ILD in people with systemic autoimmune rheumatic diseases. These guidelines will help inform clinical practice and highlight areas in which further research is needed.
间质性肺疾病（ILD）是系统性硬皮病相关间质性肺疾病（SSc-ILD）患者死亡的主要原因。美国风湿病学会（ACR）与美国胸科学会（CHEST）联合发布了系统性自身免疫性风湿病相关ILD 成人治疗的临床实践指南，其中包括 SSc-ILD。在此，我们总结了评估 SSc-ILD 治疗药物的安全性和疗效的随机试验证据。
在本综述中，我们介绍了 SSc-ILD 最近的随机对照试验结果。讨论的药物治疗包括免疫抑制剂（霉酚酸酯、环磷酰胺、利妥昔单抗和托珠单抗）和抗纤维化药物（尼达尼布和吡非尼酮）。
随机试验为 ACR/CHEST 指南中提出的 SSc-ILD 治疗建议提供了证据基础，该指南用于治疗系统性自身免疫性风湿病患者的ILD。这些指南将有助于指导临床实践，并突出需要进一步研究的领域。
