Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease
Silver, R.M.; Warrick, J.H.; Kinsella, M.B.; Staudt, L.S.; Baumann, M.H.; Strange, C.
Journal of Rheumatology 20(5): 838-844
1993
ISSN/ISBN: 0315-162X PMID: 8336309 Document Number: 408140
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 ( lt 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, p lt 0.02). Improvement was maintained at 12 months (2.27 +- 0.27 l, p lt 0.05) and 18-24 months (2.60 +- 0.28 l, p lt 0.001). 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.