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  1. Kavina Shah
  1. Correspondence to Dr Kavina Shah, Rheumatology ST4, Hammersmith Hospital, London W12 0HS, UK; kavina.shah{at}

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Cyclophosphamide for connective tissue disease-associated interstitial lung disease (CTD-ILD)

Cyclophosphamide is the accepted standard of care for individuals with progressive CTD-ILD. Its efficacy and adverse effects in CTD-ILD has recently been published in a Cochrane review (Barnes et al. Cochrane Database Syst Rev 2018;1:CD010908). Four trials with 495 participants (mostly with systemic sclerosis) were included. When compared with placebo, there was a statistically significant but clinically small improvement in post cyclophosphamide FVC (% mean difference (MD) 2.83, 95% CI 0.80 to 4.87; p=0.006) but no difference in post cyclophosphamide DLCO (% MD −1.68, 95% CI −4.37 to 1.02; p=0.22; two trials, 182 participants). When comparing cyclophosphamide with mycophenolate mofetil (MMF) at 1 year (two trials, 149 participants), there was no significant difference in lung function (p=0.61) or diffusion capacity (p=0.76). Improvement in lung function was more pronounced in patients with worse baseline spirometry and fibrosis scores. As may be expected, there was a greater risk of side effects with cyclophosphamide when compared with both placebo and …

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  • Competing interests None declared.

  • Patient consent Not required.

  • Provenance and peer review Commissioned; internally peer reviewed.

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