Journal
JOURNAL OF SCLERODERMA AND RELATED DISORDERS
Volume 5, Issue 2_SUPPL, Pages 61-71Publisher
SAGE PUBLICATIONS LTD
DOI: 10.1177/2397198320903208
Keywords
Systemic sclerosis; interstitial lung disease; clinical trials; management; treatment
Categories
Funding
- NIH/NIAMS [K24 AR063120, R01 AR-07047]
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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.
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