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Updated: Jan 23, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Impact of Evolving Treatment Patterns on Interstitial Lung Disease Progression in Systemic Sclerosis Using the
Corrado Campochiaro1, Marie-Elise Truchetet2, Madelon Vonk3
1Unit of Immunology, Rheumatology, Allergy and Rare Diseases, Inflammation, Fibrosis and Ageing Initiative, Scientific Institute for Research Hospitalization and Healthcare San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy.
Treatment for systemic sclerosis-associated interstitial lung disease (SSc-ILD) has evolved, with increased immunosuppressive therapy (IST) use. Despite advances, disease progression persists, highlighting the need for novel treatments.
Area of Science:
- Rheumatology
- Pulmonology
- Clinical Pharmacology
Background:
- Systemic sclerosis-associated interstitial lung disease (SSc-ILD) treatment has evolved with new immunosuppressive (IST) and anti-fibrotic therapies.
- Real-world utilization patterns of these therapies in SSc-ILD remain incompletely understood.
Purpose of the Study:
- To analyze temporal trends in SSc-ILD treatment strategies.
- To evaluate the impact of IST and anti-fibrotic therapies on ILD progression using the EUSTAR database.
Main Methods:
- Retrospective analysis of SSc-ILD patients from the EUSTAR database (n=1,409) meeting 2013 ACR/EULAR criteria.
- Patients were grouped into four time periods (≤2006, 2007-2011, 2012-2016, ≥2017) to assess IST initiation, switching, discontinuation, and combination therapy.
- ILD progression defined as ≥5% decline in %FVC or ≥10% decline in %DLCO over 12 months.
Main Results:
- IST use significantly increased from 13.6% to 57.4% (p<0.001), with mycophenolate mofetil becoming the most common IST.
- Combination therapy use rose from 17.9% to 26.9% (p<0.001), while ILD progression rates decreased from 21.3% to 12.1% (p<0.001).
- Factors associated with therapy initiation included shorter disease duration and myositis; switching was linked to higher mRSS and arthritis.
Conclusions:
- Clinical practice for SSc-ILD has significantly evolved, marked by increased IST utilization and combination therapy.
- Despite treatment advancements, a notable proportion of patients continue to experience disease progression, underscoring the need for more effective therapeutic strategies.
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