Abstract
IntroductionThe purpose of the present study was to systematically review the effect of cyclophosphamide treatment on pulmonary function in patients with systemic sclerosis and interstitial lung disease.MethodsThe primary outcomes were the mean change in forced vital capacity and in diffusing capacity for carbon monoxide after 12 months of therapy in patients treated with cyclophosphamide.ResultsThree randomized clinical trials and six prospective observational studies were included for analysis. In the pooled analysis, the forced vital capacity and the diffusing capacity for carbon monoxide predicted values after 12 months of therapy were essentially unchanged, with mean changes of 2.83% (95% confidence interval = 0.35 to 5.31) and 4.56% (95% confidence interval = -0.21 to 9.33), respectively.ConclusionsCyclophosphamide treatment in patients with systemic sclerosis-related interstitial lung disease does not result in clinically significant improvement of pulmonary function.
Highlights
The purpose of the present study was to systematically review the effect of cyclophosphamide treatment on pulmonary function in patients with systemic sclerosis and interstitial lung disease
Cyclophosphamide treatment in patients with systemic sclerosis-related interstitial lung disease does not result in clinically significant improvement of pulmonary function
To determine the possible benefit of CYC as management for SSc-related Interstitial lung disease (ILD), we examined the benefit of CYC on lung function as measured by pulmonary function tests by conducting a systematic review and meta-analysis of randomized clinical trials and prospective observational studies in patients with SSc treated with CYC
Summary
The purpose of the present study was to systematically review the effect of cyclophosphamide treatment on pulmonary function in patients with systemic sclerosis and interstitial lung disease. Interstitial lung disease (ILD) occurs in patients who have CREST (Calcinosis, Raynaud, ESophagitis, Telangiectases), limited cutaneous systemic sclerosis-lcSSc and diffuse cutaneous scleroderma (dcSSc), but it is somewhat more common in patients who have diffuse disease [2,3]. Pulmonary function tests with evaluation of the forced vital capacity (FVC), the total lung capacity and the diffusing lung capacity of carbon monoxide (DLCO), chest radiography and high-resolution computed tomography are common clinical tests used to evaluate lung disease in scleroderma. Previous research has found pulmonary function tests to reveal a restrictive pattern in 23% of patients with limited disease, and found 40% of patients with diffuse disease to have pulmonary fibrosis [4,5].
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