Natural variability in the disease course of SSc-ILD: implications for treatment
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...
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| Main Authors: | , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
March 24, 2021
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| In: |
European respiratory review
Year: 2021, Volume: 30, Issue: 159, Pages: 1-14 |
| ISSN: | 1600-0617 |
| DOI: | 10.1183/16000617.0340-2020 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1183/16000617.0340-2020 Verlag, lizenzpflichtig, Volltext: https://err.ersjournals.com/content/30/159/200340 
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| Author Notes: | Madelon C. Vonk, Ulrich A. Walker, Elizabeth R. Volkmann, Michael Kreuter, Sindhu R. Johnson and Yannick Allanore |
| Summary: | 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. </p><p>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.</p><p>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. |
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| Item Description: | Gesehen am 09.07.2021 |
| Physical Description: | Online Resource |
| ISSN: | 1600-0617 |
| DOI: | 10.1183/16000617.0340-2020 |