University of Cincinnati, Cincinnati, OH, USA.
Houston Methodist Hospital, Houston, TX, USA.
Curr Rheumatol Rep. 2022 May;24(5):166-173. doi: 10.1007/s11926-022-01067-5. Epub 2022 May 1.
Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc). We explore the importance of early detection, monitoring, and management of SSc-ILD.
All patients with SSc are at risk of ILD and should be screened for ILD at diagnosis using a high-resolution computed tomography (HRCT) scan. Some patients with SSc-ILD develop a progressive phenotype characterized by worsening fibrosis on HRCT, decline in lung function, and early mortality. To evaluate progression and inform treatment decisions, regular monitoring is important and should include pulmonary function testing, evaluation of symptoms and quality of life, and, where indicated, repeat HRCT. Multidisciplinary discussion enables comprehensive evaluation of the available information and its implications for management. The first-line treatment for SSc-ILD is usually immunosuppression. The antifibrotic drug nintedanib has been approved for slowing lung function decline in patients with SSc-ILD. Optimal management of patients with SSc-ILD requires a multidisciplinary and patient-centered approach.
间质性肺疾病(ILD)是系统性硬化症(SSc)的常见表现。我们探讨了早期发现、监测和管理 SSc-ILD 的重要性。
所有 SSc 患者均有发生 ILD 的风险,应在诊断时使用高分辨率计算机断层扫描(HRCT)对 ILD 进行筛查。一些 SSc-ILD 患者会出现进行性表型,其特征是 HRCT 上纤维化恶化、肺功能下降和早期死亡率增加。为了评估进展并为治疗决策提供信息,定期监测很重要,应包括肺功能测试、症状和生活质量评估,以及在有指征时重复 HRCT。多学科讨论可对现有信息进行全面评估,并了解其对管理的影响。SSc-ILD 的一线治疗通常是免疫抑制。抗纤维化药物尼达尼布已被批准用于减缓 SSc-ILD 患者的肺功能下降。优化 SSc-ILD 患者的管理需要多学科和以患者为中心的方法。