Patients with scleroderma, systemic sclerosis (SSc), myositis and rheumatoid arthritis (RA) may develop interstitial lung disease (ILD), which affects a patient’s breathing and quality of life. Prospective studies have revealed that in patients with SSc a greater rate of decline of forced vital capacity (FVC) is associated with increased mortality. Although corticosteroids are commonly used to treat these patients, little evidence supports this approach. Alternatively, physicians may prescribe cyclophosphamide to treat patients with severe disease and/or rapidly progressing disease even though it is associated with multiple side effects and toxicities.
Explore this issueMay 2018
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Cochrane Library Review
A new Cochrane Library review by Hayley Barnes, MBBS, a pulmonologist at Monash University in Australia, and colleagues, evaluated whether cyclophosphamide can help preserve lung function in patients with ILD due to connective tissue disease. The authors also examined whether cyclophosphamide helps improve quality of life, length of life, breathing and ability to exercise, as well as the cost at which the treatment achieves these benefits.1
The team found that patients with ILD associated with connective tissue disease may realize a small benefit from cyclophosphamide treatment. Unfortunately, because connective tissue disease-associated ILD is a rare lung disease, research in this area is challenged by the small number of potential study participants.