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Efficacy of Cyclophosphamide in the Treatment of Interstitial Lung Disease Associated with Systemic Sclerosis
Authors:Gerard Espinosa  Carmen Pilar Simeon  Miguel Angel Plasin  Antoni Xaubet  Xavier Muñoz  Vicent Fonollosa  Ricard Cervera  Miquel Vilardell
Institution:1. Department of Medicine, Division of Pulmonary, Allergy and Critical Care Medicine, Boston University School of Medicine, Boston, MA;2. Biostatistics Core, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA;3. Department of Biomathematics, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA;4. Department of Medicine, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA
Abstract:BackgroundCyclophosphamide (CYC) stabilizes the parameters of lung function tests (LFT) of patients with systemic sclerosis (SSc) and interstitial lung disease (ILD) treated for 12 months. There is little information about long-term treatment (24 months). The aim of this study is to analyze the effect of intravenous CYC in LFT parameters in patients with SSc and ILD treated for 24 months.Patients and methodRetrospective study of 37 patients with ILD associated with scleroderma treated with intravenous CYC for 24 months and regularly assessed by LFT (at baseline, 6, 12 and 24 months) including forced vital capacity (FVC) and transfer capacity of carbon monoxide (DLCO).ResultsThe differences between FVC and DLCO values performed at baseline and those performed at 6, 12, and 24 months were less than 10%, which meant that CYC stabilized functional parameters. There were no differences in FVC or DLCO when patients treated for 6 months were evaluated according to the type of SSc skin involvement of (diffuse or limited) or according to the evolution time of ILD before the start of treatment. Although patients with severe restriction (FVC<70%) showed more improvement, it was less than 10% in all casesConclusionIn this series of patients with ILD associated with SSc, intravenous CYC was effective in stabilizing lung function parameters in long-term treatment.
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