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Updated: Jul 5, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Oral cyclophosphamide for active scleroderma lung disease: a decision analysis
Dinesh Khanna1, Daniel E Furst, Philip J Clements
1Division of Rheumatology, School of Public Health, University of California at Los Angeles, Los Angeles, CA 90095, USA. dkhanna@mednet.ucla.edu
One year of cyclophosphamide (CYC) for scleroderma-related interstitial lung disease (SSc-ILD) may lead to a slight decrease in quality-adjusted life years (QALYs). This finding considers CYC
Area of Science:
- Rheumatology
- Pulmonary Medicine
- Health Economics
Background:
- Scleroderma Lung Study (SLS) indicated oral cyclophosphamide (CYC) delays scleroderma-related interstitial lung disease (SSc-ILD) progression at 12 months.
- However, CYC is linked to adverse events, and its long-term risk-benefit balance for SSc-ILD remains unclear.
Purpose of the Study:
- To evaluate the risk-benefit tradeoffs of cyclophosphamide (CYC) versus no specific therapy for patients with scleroderma-related interstitial lung disease (SSc-ILD).
- To project quality-adjusted life years (QALYs) using a Markov decision analytic model.
Main Methods:
- A Markov decision analytic model was employed to assess QALYs for CYC versus no CYC treatment in SSc-ILD.
- The base case analyzed a 50-year-old woman with SSc and moderate SSc-ILD, comparing 1 year of daily CYC to no SSc-ILD-specific therapy.
- Sensitivity analyses explored variations in CYC efficacy and survival benefits.
Main Results:
- In the base-case analysis, CYC treatment resulted in a small loss of 0.21 QALYs (16.84 vs. 17.15).
- CYC was found to be inferior across most sensitivity analyses.
- However, analyses incorporating a survival benefit for CYC showed a significant gain in QALYs (18.17 vs. 17.15).
Conclusions:
- One year of CYC therapy for SSc-ILD leads to a minor decrease in QALYs compared to no treatment.
- This conclusion is driven by the lack of a survival benefit and the transient effect of CYC on pulmonary function.
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