Pharmacological treatment of
diffuse systemic sclerosis (SSc) directed at the tissue
fibrosis has generally been ineffective. Many immunosuppressive drugs have been tried as
therapy for SSc, regardless of the disease subtype and/or stage. The aim of this study was to show the efficacy and the toxicity of oral
cyclophosphamide and
prednisolone therapy on the prevention of
fibrosis-based tissue damage in the early stages of the diffuse SSc. Twenty-seven patients with early diffuse SSc were treated with oral
cyclophosphamide (1-2 mg/kg/day) plus oral
prednisolone (40 mg/every other day) between the years 1995 and 1998. The results regarding the efficacy and toxicity of
cyclophosphamide were compared with those of 22 early SSc patients who had been treated with oral
D-penicillamine between 1992 and 1995. All the patients were evaluated using clinical and laboratory parameters every 6 months for 2 years. There was a significant improvement on the skin score, maximal oral opening, flexion index, predicted forced vital capacity (FVC) and
carbon monoxide diffusing capacity (DLCO) in the
cyclophosphamide group. The decrease in skin score in the
cyclophosphamide group started earlier than in the
D-penicillamine group. No life-threatening or irreversible adverse reaction was observed. This open study supports the use of oral
cyclophosphamide plus
prednisolone therapy to prevent
fibrosis and its complications in the early stages of diffuse SSc.