Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease.
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, p < 0.02). Improvement was maintained at 12 months (2.27 +/- 0.27 l, p < 0.05) and 18-24 months (2.60 +/- 0.28 l, p < 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.
Silver
R M
RM
Department of Medicine, Medical University of South Carolina, Charleston 29425.
Warrick
J H
JH
Kinsella
M B
MB
Staudt
L S
LS
Baumann
M H
MH
Strange
C
C
D000328:Adult; D001992:Bronchoalveolar Lavage Fluid / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage* / Q000627:therapeutic use; D011446:Prospective Studies; D011653:Pulmonary Diffusing Capacity; D011658:Pulmonary Fibrosis / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000188:drug therapy*; D014797:Vital Capacity
D003520:Cyclophosphamide; D011241:Prednisone