The efficacy of oral cyclophosphamide plus prednisolone in early diffuse systemic sclerosis

Abstract

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.

Corticosteroids and the risk of scleroderma renal crisis: a systematic review

“…The two possible cases had both received antithymocyte globulin. Considering only the 11 studies limited to early dcSSc patients [18,21,22,25,27,28,30,31,33,38,49], we identified 9 definite SRC cases in 226 patients, which is equivalent to a rate of 4%.…”

Targeting activated T cells: successful use of anti-CD25 monoclonal antibody basiliximab in a patient with systemic sclerosis

“…Some studies showed that early immunosuppressive treatment with high-dose corticosteroids and pulse intravenous cyclophosphamide results in good cardiac outcome in acute lupus myocarditis. 10 This case report highlights the therapeutic benefit of ASCT in the treatment of sclerodermatous myocarditis, and in scleroderma affecting skin, tendon sheaths and skin in a patient with early diffuse scleroderma positive for exposure to silica.…”

“…7 More prominent effects have been described in patients with early SSc treated with oral CYC. 10 The case presented points towards a beneficial effect of targeting activated T cells in skin invlovement in SSc, especially early in the course of disease. C ardiac involvement in scleroderma is often subclinical [1][2][3] and occurs early in the disease.…”

Low influenza vaccination rate among patients with systemic sclerosis

“…The results of two recent, prospective, randomized trials do not favour the use of CYC for SSc-associated ILD [31,32], although limited benefit was observed in patients treated with oral CYC as compared with placebo in the scleroderma lung study [31]. In addition, open and/or retrospective studies of patients with early diffuse SSc revealed an improved Rodnan skin score on treatment with CYC [33,34] and mycophenolate mofetil [35]. In the present study, 42 (23.7%) of the 177 SSc patients were receiving ISs.…”