Dept of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands.
Dept of Rheumatology, University Hospital Basel, Basel, Switzerland.
Eur Respir Rev. 2021 Mar 24;30(159). doi: 10.1183/16000617.0340-2020. Print 2021 Mar 31.
Interstitial lung disease (ILD) affects approximately 50% of patients with systemic sclerosis (SSc) and is the leading cause of death in SSc. Our objective was to gain insight into the progression of SSc-associated ILD (SSc-ILD). Using data from longitudinal clinical trials and observational studies, we assessed definitions and patterns of progression, risk factors for progression, and implications for treatment. SSc-ILD progression was commonly defined as exceeding specific thresholds of lung function worsening and/or increasing radiographic involvement. One definition used in several studies is decline in forced vital capacity (FVC) of ≥10%, or ≥5-10% plus a decline in diffusing capacity of the lung for carbon monoxide ≥15%. Based on these criteria, 20-30% of patients in observational cohorts develop progressive ILD, starting early in the disease course and progressing at a highly variable rate.Risk factors such as age, FVC, extent of fibrosis and presence of anti-topoisomerase I antibodies can help predict progression of SSc-ILD, though composite risk scores may offer greater predictive power. Whilst the variability of the disease course in SSc-ILD makes risk stratification of patients challenging, the decision to initiate, change or stop treatment should be based on a combination of the current disease state and the speed of progression.
间质性肺疾病（ILD）影响约 50%的系统性硬化症（SSc）患者，是 SSc 患者死亡的主要原因。我们的目的是深入了解与 SSc 相关的 ILD（SSc-ILD）的进展情况。我们利用来自纵向临床试验和观察性研究的数据，评估了进展的定义和模式、进展的危险因素以及对治疗的影响。SSc-ILD 进展通常定义为超过特定的肺功能恶化和/或放射学受累增加的阈值。有几项研究中使用的一个定义是用力肺活量（FVC）下降≥10%，或≥5-10%加一氧化碳弥散量下降≥15%。根据这些标准，观察队列中 20-30%的患者出现进行性 ILD，从疾病早期开始，并以高度可变的速度进展。年龄、FVC、纤维化程度和抗拓扑异构酶 I 抗体等危险因素有助于预测 SSc-ILD 的进展，尽管复合风险评分可能具有更大的预测能力。虽然 SSc-ILD 疾病过程的变异性使得对患者进行风险分层具有挑战性，但启动、改变或停止治疗的决定应基于当前疾病状态和进展速度的结合。
