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Fourteen patients with systemic sclerosis (SSc, scleroderma) and interstitial lung disease were treated with oral cyclophosphamide (1-2 mg/kg/day) and low dose prednisone (10 mg/day). There was a significant improvement in FVC after 6 months compared to entry values (2.21 +/- 0.19 l vs. 2.03 +/- 0.15 l, p0.02). Improvement was maintained at 12 months (2.27 +/- 0.27 l, p0.05) and 18-24 months (2.60 +/- 0.28 l, p0.001). In 12 cases followed for 18-24 months, FVC was stable or improved. No significant improvement or decline was noted for the DLCO. Side effects included cytopenia (2), infection (1), and hemorrhagic cystitis (2), and one possible related malignancy. A controlled prospective trial of cyclophosphamide is warranted in patients with SSc and active interstitial lung disease.

Seventeen patients with early systemic sclerosis (SSc) underwent high resolution computed tomography (HRCT) of the chest to evaluate dyspnea and/or abnormal pulmonary function tests (PFT). All patients were assigned a dyspnea score and each had routine chest radiography (CXR). Bronchoalveolar lavage (BAL) was performed on 10 patients. HRCT was abnormal in 15 patients (88%), while CXR was abnormal in only 10 patients (59%). Mediastinal lymphadenopathy was detected in 7 patients (41%). Disease duration, dyspnea score, and forced vital capacity (FVC) did not correlate with HRCT score. However, trends toward higher total BAL cell counts and higher BAL neutrophil counts were noted in patients with ground glass opacities on HRCT, and BAL lymphocyte counts were significantly higher in such cases. HRCT is superior to CXR for detecting early interstitial lung disease in SSc, but patient history and FVC correlate poorly with HRCT findings. Ground glass opacities on HRCT may reflect active alveolitis, and mediastinal lymphadenopathy associated with SSc lung disease may be a consequence of pulmonary inflammation.