Combination of intravenous pulses of cyclophosphamide and methylprednizolone in patients with systemic sclerosis and interstitial lung disease.

The purpose of the study was to examine prospectively the efficacy and safety of the combination of intravenous pulses of cyclophosphamide and methylprednizolone, in the treatment of scleroderma lung disease. Thirteen patients were treated with the above combination for up to 24 months. Prior to this treatment, they underwent a pulmonary function evaluation and high resolution computed tomography (HRCT). Carbon monoxide diffusion lung capacity and forced vital capacity were repeated at 6, 12, 24 and 48 months. HRCT was repeated at the end of the treatment period, but in between and afterwards in some patients, as well. A significant percentage of patients (66.6%) showed stabilization or improvement of their pulmonary function. Patients with already seriously compromised function, before treatment, were the least likely to exhibit this evolution pattern. There was a tendency in some individuals to deteriorate on later evaluations, off treatment, although they had stabilized at the end of the treatment. There was rather a poor correlation between functional evolution and HRCT appearance. Finally, the regimen was well tolerated. Our results suggest that the employed combination is safe and effective, mainly in stabilizing the respiratory function of the patients. This goal is more realistic when treatment is given before significant functional compromise has ensued. The need for long-term immunosuppression to maintain the initial favorable response is suggested.