In the past years renal crisis was the major cause of morbidity and lethal outcome in patients with scleroderma (SSc). Today, after the introduction into to medical practice of angiotensin-converting enzyme inhibitors used in the treatment of renovascular hypertension, pulmonary fibrosis and pulmonary hypertension have become the most common causes of death in SSc. As a result, the early diagnosis and treatment of pulmonary fibrosis is essential to decrease death rates and morbidity in SSc patients. The purpose of this study was to analyze the efficacy of intravenous cyclophoshamide pulse therapy in patients with SSc and the proof of active alveolitis assessed on a high resolution computed tomographic (HRCT) scan, and to compare the effectiveness of cyclophosphamide pulse therapy with oral therapy. Sixteen SSc patients were divided alternately into the two test groups. Eight patients have undergone monthly cyclophoshamide pulse therapy (750 mg/m2) for 12 months; the patients from the second group were given with oral cyclophosphamide (2-2.5 mg/kg/day) for the same period of time. All the patients involed in the study were given concurrently prednisone (10 mg/day). HRCT scans an d pulmonary function tests were done before the treatment and at 6 and 12 months. In the oral cyclophosphamide patient group, three patients with a grade I pattern experienced regression of disease extent. In the five remaining patients (one with grade II and four with grade III) the pattern and extent of disease stayed stable in the course of the research. The diffusing capacity for carbon monoxide increased dramatically between baseline and 12 months (p = 0.043). No statistical disparities were found in forced expiratory volume in 1 s, forced vital capacity and overall lung capacity during the study period. In the cyclophosphamide pulse therapy group, seven patients with a grade I pattern experienced disease regression at 6 months (p = 0.018) and 12 months (p = 0.012). One patient with grade III was stable during the research. In both groups the regression of the extent of disease evaluated on HRCT was due to the lessening in the ground glass appearance. The degree of the reticular appearance stayed stable throughout the research. Our findings imply that cyclophosphamide pulse therapy is effective in suppressing active alveolitis (ground glass appearance). Although this research is not enough to compare pulse therapy with oral therapy because of the pattern difference observed on HRCT between the two patient groups, oral therapy may be effective in suppressing active alveolitis too. Neither regimen improved pulmonary involvement when the reticular appearance predominated over the ground glass appearance on HRCT. The conclusion runs as follows: either pulse or oral cyclophosphamide therapy is beneficial for SSc patients and diminished the chance or lethal outcome.

barryCyclophosphamide Researches7 Mar, 2010