ArticleRMD open2024
Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort.
Article in RMD open, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed.
- Review
- Evolving landscape of imaging-based evaluation in systemic autoimmune rheumatic disease-associated interstitial lung disease: from visual assessment to quantitative artificial intelligence-assisted evaluation.Journal of rheumatic diseases · 2026Review
- Associations with, and predictors of, progression in systemic sclerosis-related interstitial lung disease: a scoping literature review.European respiratory review : an official journal of the European Respiratory Society · 2026Article
- Machine Learning Analysis of Electronic Health Records Identifies Interstitial Lung Disease and Predicts Mortality in Patients with Systemic Sclerosis.medRxiv : the preprint server for health sciences · 2025Article
- Understanding Late-Onset Interstitial Lung Disease in Systemic Sclerosis: Implications for Clinical Practice and Trial Design.Arthritis & rheumatology (Hoboken, N.J.) · 2025Article
- A scoping review of the epidemiology of systemic sclerosis and its organ manifestations: 2018-2024.Current opinion in rheumatology · 2025Article
- Circulating extracellular vesicles as predictive biomarkers of progressive interstitial lung disease in systemic sclerosis-a prospective cohort study.Frontiers in medicine · 2025Article
Corrections and comments
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Authors and funding
15 authors.
Funding
Abstract
backgroundInterstitial lung disease (ILD) is the leading cause of death in systemic sclerosis (SSc). According to expert statements, not all SSc-ILD patients require pharmacological therapy.
objectivesTo describe disease characteristics and disease course in untreated SSc-ILD patients in two well characterised SSc-ILD cohorts.
methodsPatients were classified as treated if they had received a potential ILD-modifying drug. ILD progression in untreated patients was defined as (1) decline in forced vital capacity (FVC) from baseline of ≥10% or (2) decline in FVC of 5%-9% associated with a decline in diffusing capacity for carbon monoxide (DLCO)≥15% over 12±3 months or (3) start of any ILD-modifying treatment or (4) increase in the ILD extent during follow-up. Multivariable logistic regression was performed to identify factors associated with non-prescription of ILD-modifying treatment at baseline. Prognostic factors for progression in untreated patients were tested by multivariate Cox regression.
resultsOf 386 SSc-ILD included patients, 287 (74%) were untreated at baseline. Anticentromere antibodies (OR: 6.75 (2.16-21.14), p=0.001), limited extent of ILD (OR: 2.39 (1.19-4.82), p=0.015), longer disease duration (OR: 1.04 (1.00-1.08), p=0.038) and a higher DLCO (OR: 1.02 (1.01-1.04), p=0.005) were independently associated with no ILD-modifying treatment at baseline. Among 234 untreated patients, the 3 year cumulative incidence of progression was 39.9% (32.9-46.2). Diffuse cutaneous SSc and extensive lung fibrosis independently predicted ILD progression in untreated patients.
conclusionAs about 40% of untreated patients show ILD progression after 3 years and effective and safe therapies for SSc-ILD are available, our results support a change in clinical practice in selecting patients for treatment.
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