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Open Access Highly Accessed Research article

Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies

Carlotta Nannini1*, Colin P West23, Patricia J Erwin4 and Eric L Matteson1

Author Affiliations

1 Division of Rheumatology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA

2 Division of General Internal Medicine, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA

3 Division of Biostatistics, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA

4 Medical Library, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA

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Arthritis Research & Therapy 2008, 10:R124  doi:10.1186/ar2534


See related editorial by Miniati et al., http://arthritis-research.com/content/11/1/103

Published: 20 October 2008

Abstract

Introduction

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.

Methods

The 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.

Results

Three 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.

Conclusions

Cyclophosphamide treatment in patients with systemic sclerosis-related interstitial lung disease does not result in clinically significant improvement of pulmonary function.