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Outcome of systemic sclerosis associated interstitial lung disease treated with intravenous cyclophosphamide
Clinical Rheumatology, 04/15/2011
Clinical Article
Abhishek A et al. –The objective of this study was to audit the respiratory outcome, toxicity and long-term survival of systemic sclerosis associated interstitial lung disease (SSc-ILD) treated with intravenous (i.v.) cyclophosphamide. I.V. cyclophosphamide stabilises lung function in individuals with SSc-ILD and may associate with better respiratory outcome in patients diagnosed on screening PFTs.
Methods- Ascertained whether i.v. cyclophosphamide associates with better outcome in SSc-ILD diagnosed due to decline in screening lung function than in those diagnosed due to respiratory symptoms
- Retrospective case-note audit
- Carried out for SSc-ILD patients treated with i.v. cyclophosphamide between January 1999 and March 2009 at Royal Derby, Kings Mill and Nottingham University Hospitals
- Forced vital capacity (FVC) and transfer factor at 6, 12 months after starting i.v. cyclophosphamide were primary end points
- Kaplan–Meier curves plotted to estimate survival
- 37 i.v. cyclophosphamide treatment cycles administered to 36 patients (27 women)
- 14 cycles associated with SE and 8 terminated prematurely
- SSc-ILD diagnosed due to respiratory symptoms in 13 and in response to deteriorating screening pulmonary function test (PFT) in 24 instances
- Overall, i.v. cyclophosphamide led to stabilisation in lung function
- FVC declined by 7% in SSc-ILD presenting with respiratory symptoms over 12 months
- Significantly lower FVC at 6 and 12 month than those with SSc-ILD diagnosed due to decline in screening lung function
- 5-year survival 76.1% (overall), 62.9% (diagnosed due to respiratory symptoms) and 91.5% (diagnosed due to decline in screening lung function)
