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Journal Articles European Respiratory Review Year : 2021

Natural variability in the disease course of SSc-ILD: implications for treatment

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Abstract

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.

Dates and versions

hal-03258403 , version 1 (11-06-2021)

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Madelon Vonk, Ulrich Walker, Elizabeth Volkmann, Michael Kreuter, Sindhu Johnson, et al.. Natural variability in the disease course of SSc-ILD: implications for treatment. European Respiratory Review, 2021, 30 (159), pp.200340. ⟨10.1183/16000617.0340-2020⟩. ⟨hal-03258403⟩

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