Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis

Background: Lung inflammation (alveolitis) may cause lung fibrosis in scler oderma. Objective: To determine whether cyclophosphamide treatment is associated wi th retention of lung function and improved survival in scleroderma patients with alveolitis. Design: Retrospective cohort study, Setting: Johns Hopkins and University o f Maryland Scleroderma Center. Patients: 103 patients with scleroderma who had bronchoalveolar lavage or l ung biopsy. Intervention: Cyclophosphamide therapy. Measurements: 1) Serial measurement of forced vital capacity (FVC)and carbo n monoxide diffusing capacity and 2) survival. Results: During a median follow-up of 13 months after bronchoalveolar ravag e or biopsy, patients with alveolitis who did not receive cyclophosphamide therapy experienced a decrease in FVC (mean difference, -0.28 L [95% Cl, -0 .41 to -0.16 L] and -7.1% of the predicted value [CI, -10.9% to -4.0%]). Ca rbon monoxide diffusing capacity also decreased in these patients (mean dif ference, -3.3 mmol min(-1).kPa(-1) [Cl, -4.6 to -2.1 mmol.min(-1).kPa(-1)] and -9.6% of the predicted value [CI, -16.7% to -2.4%]). During a median fo llow-up of 16 months, patients with alveolitis who received cyclophosphamid e we re more likely to have a good outcome (stabilization or improvement) i n FVC (relative risk, 2.5 [CI, 1.5 to 4.1]) and diffusing rapacity (relativ e risk, 1.5 [CI, 1.0 to 2.2]). These patients also had improved survival; t he median survival rate was 89% (25th, 75th percentiles, 84%, 94%) compared with 71% (25th, 75th percentiles, 55%, 86%) in untreated patients (P = 0.0 1, log-rank test). Conclusions: The presence of lung inflammation identifies patients with scl eroderma who are more likely to have worsening lung function. Lung function outcomes and survival are improved in patients with alveolitis who receive cyclophosphamide.