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Updated: Jul 18, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and
B White1, W C Moore, F M Wigley
1Veterans Affairs Maryland Health Care System, University of Maryland School of Medicine, and Johns Hopkins University Medical Institutions, Baltimore, USA.
Cyclophosphamide treatment improved lung function and survival in scleroderma patients with alveolitis (lung inflammation). This therapy helps stabilize or improve forced vital capacity and diffusing capacity, offering better outcomes for patients.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Scleroderma can lead to lung inflammation (alveolitis), a precursor to lung fibrosis.
- Alveolitis significantly impacts lung function and patient survival in scleroderma.
Purpose of the Study:
- To investigate the association between cyclophosphamide treatment and lung function retention in scleroderma patients with alveolitis.
- To determine if cyclophosphamide therapy improves survival rates in this patient cohort.
Main Methods:
- Retrospective cohort study involving 103 scleroderma patients with confirmed alveolitis.
- Evaluated the impact of cyclophosphamide therapy on serial measurements of forced vital capacity (FVC) and carbon monoxide diffusing capacity.
- Survival analysis was conducted to compare outcomes between treated and untreated patients.
Main Results:
- Patients with alveolitis not receiving cyclophosphamide showed significant decreases in FVC and diffusing capacity.
- Cyclophosphamide-treated patients demonstrated a higher likelihood of stabilized or improved FVC (RR, 2.5) and diffusing capacity (RR, 1.5).
- Median survival rates were significantly higher in the cyclophosphamide group (89%) compared to the untreated group (71%, P=0.01).
Conclusions:
- Alveolitis is a key indicator of potential lung function decline in scleroderma patients.
- Cyclophosphamide therapy is associated with improved lung function and enhanced survival in scleroderma patients suffering from alveolitis.
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