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Natural variability in the disease course of SSc-ILD: implications for treatment
European Respiratory Review ( IF 9.0 ) Pub Date : 2021-03-24 , DOI: 10.1183/16000617.0340-2020
Madelon C Vonk 1 , Ulrich A Walker 2 , Elizabeth R Volkmann 3 , Michael Kreuter 4 , Sindhu R Johnson 5 , Yannick Allanore 6
Affiliation  

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.



中文翻译:

SSc-ILD 病程的自然变异性:对治疗的影响

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。

更新日期:2021-03-24
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