RT Journal Article SR Electronic T1 Natural variability in the disease course of SSc-ILD: implications for treatment JF European Respiratory Review JO EUROPEAN RESPIRATORY REVIEW FD European Respiratory Society SP 200340 DO 10.1183/16000617.0340-2020 VO 30 IS 159 A1 Madelon C. Vonk A1 Ulrich A. Walker A1 Elizabeth R. Volkmann A1 Michael Kreuter A1 Sindhu R. Johnson A1 Yannick Allanore YR 2021 UL http://err.ersjournals.com/content/30/159/200340.abstract AB 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.The disease course of SSc-ILD is heterogeneous and variable. More data are needed to better understand the natural disease course of SSc-ILD, enabling risk stratification of patients to optimise the management and long-term outcomes. https://bit.ly/3qqITaZ