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Updated: Sep 28, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Treatment of systemic sclerosis-associated interstitial lung disease: Lessons from clinical trials
David Roofeh1, Oliver Distler2, Yannick Allanore3
1Scleroderma Program, University of Michigan, Ann Arbor, MI, USA.
Abstract:
Systemic sclerosis-associated interstitial lung disease remains a leading cause of mortality. Despite decades of clinical trials, the treatment effects of disease modifying anti-rheumatic drugs continue to be modest and there remains a great need for therapies that attenuate and hopefully ameliorate parenchymal lung disease. In this review, we highlight the key clinical trials that have shaped the management strategies employed by the authors, providing their strength of recommendation based on level of evidence. We also review lessons learned in more recent years, suggesting a benefit in targeting patients with subclinical interstitial lung disease with high risk for progression early in the disease course, as well as the benefit seen in a large clinical trial leading to the first Food and Drug Administration-approved treatment for systemic sclerosis-associated interstitial lung disease. These lessons come in a context of heterogeneity of patient populations and response to therapy, as well as the inherent constraints of time-limited studies to detect meaningful outcomes for patients.
Insights
Systemic sclerosis-associated interstitial lung disease (SSc-ILD) treatments need improvement. Early intervention and new FDA-approved therapies offer hope for managing this condition and improving patient outcomes.
Area of Science:
- Rheumatology
- Pulmonology
- Clinical Trials
Background:
- Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a major cause of mortality.
- Current disease-modifying anti-rheumatic drugs (DMARDs) offer modest benefits.
- There is a critical need for novel therapies to treat SSc-ILD.
Purpose of the Study:
- To review key clinical trials in SSc-ILD management.
- To provide evidence-based recommendations for SSc-ILD treatment strategies.
- To discuss recent advancements and lessons learned in SSc-ILD therapy.
Main Methods:
- Review of pivotal clinical trials in SSc-ILD.
- Analysis of treatment effects and levels of evidence.
- Synthesis of recent findings and clinical trial outcomes.
Main Results:
- DMARDs have shown limited efficacy in treating SSc-ILD.
- Early targeted therapy for high-risk patients with subclinical SSc-ILD shows promise.
- A recent large clinical trial led to the first FDA-approved treatment for SSc-ILD.
Conclusions:
- Management strategies for SSc-ILD are evolving.
- Personalized treatment approaches considering patient heterogeneity are crucial.
- Future research should address limitations of time-limited studies for long-term outcome assessment.
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