系统性硬皮病相关间质性肺疾病的治疗。
Treatment for systemic sclerosis-associated interstitial lung disease.
机构信息
Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA.
Department of Internal Medicine and Clinical Immunology, Rennes University Hospital.
出版信息
Curr Opin Rheumatol. 2021 May 1;33(3):240-248. doi: 10.1097/BOR.0000000000000795.
PURPOSE OF REVIEW
This review provides an overview of the current treatments for systemic sclerosis-interstitial lung disease (SSc-ILD) and proposes a conceptual framework for disease management with case scenarios.
RECENT FINDINGS
Broad treatment categories include traditional cytotoxic therapies, biologic disease-modifying rheumatic drugs, antifibrotic agents, autologous hematopoietic stem cell transplant, and lung transplantation. The optimal use of each option varies depending on SSc-ILD severity, progression, and comorbidities of individual patients. A high-quality randomized controlled trial demonstrated nintedanib's ability to retard decline of lung function in patients with limited and diffuse cutaneous disease, with established ILD. Tocilizumab, recently approved by the FDA, provides a unique intervention in those with early SSc associated with ILD with elevated acute-phase reactants: two well designed trials showed lung function preservation in phase 2 and phase 3 trials.
SUMMARY
Stratifying patients based on key SSc-ILD characteristics (e.g. severity, risk of progression, comorbid disease presentation) may provide a useful guide for practitioners treating SSc-ILD.
目的综述
本文概述了目前系统性硬化症间质性肺病（SSc-ILD）的治疗方法，并通过病例场景提出了疾病管理的概念框架。
最新发现
广泛的治疗类别包括传统细胞毒性疗法、生物性疾病修饰抗风湿药物、抗纤维化药物、自体造血干细胞移植和肺移植。每种选择的最佳使用取决于 SSc-ILD 的严重程度、进展和个体患者的合并症。一项高质量的随机对照试验证明尼达尼布能够延缓局限性和弥漫性皮肤疾病合并已确诊ILD 患者的肺功能下降。托珠单抗最近被 FDA 批准，为伴有ILD 和急性期反应物升高的早期 SSc 患者提供了一种独特的干预措施：两项精心设计的试验显示，在 2 期和 3 期试验中可保留肺功能。
总结
根据 SSc-ILD 的关键特征（如严重程度、进展风险、合并症表现）对患者进行分层，可能为治疗 SSc-ILD 的医生提供有用的指导。
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