Objectives: To determine whether cyclophosphamide is beneficial for patients with scleroderma lung disease (SLD).
Methods: The effect of 6 months' treatment with intravenous cyclophosphamide on the functional capacity of patients, lung function tests, high resolution computed tomography of the lungs, and cytology of bronchoalveolar lavage was evaluated in 21 patients with SLD.
Results: The treatment was well tolerated and all patients completed 6 months' treatment. Intravenous cyclophosphamide stabilised or improved the patients' functional status and lung function tests. The extent of the lungs affected remained unchanged, as assessed with HRCT of the lungs. Patients with SLD and neutrophilic alveolitis (NA) showed greater improvement than patients with normal levels of granulocytes in the bronchoalveolar lavage fluid (BALF). Significant reduction of neutrophils was also seen in the patients with SLD and NA, whereas no significant change was seen in the level of granulocytes in patients with SLD and an initially normal percentage of granulocytes.
Conclusions: Previous reports that patients with SLD with increased levels of granulocytes in BALF are more likely to benefit from treatment with intravenous cyclophosphamide are confirmed. Additionally, clinical improvement in this group of patients is accompanied by a significant decrease in the percentage of granulocytes in BALF.