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.

间质性肺病 (ILD) 影响大约 50% 的系统性硬化症 (SSc) 患者，并且是 SSc 死亡的主要原因。我们的目标是深入了解 SSc 相关 ILD (SSc-ILD) 的进展。使用来自纵向临床试验和观察性研究的数据，我们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 进展通常被定义为超过肺功能恶化和/或放射学受累增加的特定阈值。多项研究中使用的一个定义是用力肺活量 (FVC) 下降 ≥ 10%，或 ≥ 5–10% 加上肺一氧化碳扩散能力下降 ≥ 15%。根据这些标准，观察队列中 20-30% 的患者发展为进行性 ILD，在病程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 抗体的存在等风险因素可以帮助预测 SSc-ILD 的进展，尽管综合风险评分可能提供更大的预测能力。虽然 SSc-ILD 病程的可变性使患者的风险分层具有挑战性，但开始、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组合。