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. 2011 Jan 13:5:1-6.
doi: 10.2174/1874312901105010001.

Long term effects of cyclophosphamide treatment on lung function and survival in scleroderma patients with interstitial lung disease

S Mittoo  1 , Fredrick M Wigley, Robert A Wise, Adrianne Woods, Huiqing Xiao, Laura K Hummers
Affiliations

Long term effects of cyclophosphamide treatment on lung function and survival in scleroderma patients with interstitial lung disease

S Mittoo et al. Open Rheumatol J. .

Abstract

Background: Scleroderma (SSc) patients with active interstitial lung disease (ILD) experience a decline in lung function and increased mortality; cyclophosphamide (CYC) therapy may stabilize lung function at one and two years follow-up. Long-term lung function and survival outcomes of SSc patients with ILD following CYC treatment remain largely unknown.

Methodology: We reviewed records of SSc patients with active ILD who had received at least six months of CYC treatment and had pulmonary function tests (PFTs) performed at least two years from the onset of treatment.

Principal findings: Thirty eight patients meeting eligibility criteria had a mean follow-up period from start of CYC to the last follow-up PFT of 5.1 years (range 2.3 -10.8 years). At a median of 4.1 years (range 9 months - 8.4 years), 12/38 (32%) patients had a significant decline in % predicted Forced Vital Capacity from their baseline PFT. At a median of 3.9 years (range 7 months - 8.4 years); 12/36 (33%) patients experienced a significant decline in their % predicted carbon monoxide diffusing capacity. Three patients died at a follow-up between 4.5-6 years and two received bilateral lung transplants because of severe restrictive lung disease.

Conclusions: While the majority of SSc patients treated with CYC for active ILD experience long-term lung function stability and survive, greater than 1/3 of patients will experience either lung function decline, death, or require a lung transplant. This suggests that despite aggressive immune suppressing therapy, a subset of patients will have continued lung function decline, highlighting the need for ongoing monitoring and better therapeutic options.

Keywords: Scleroderma; cyclophosphamide.; pulmonary fibrosis.

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Figures

Fig. (1)
Fig. (1)
Kaplan-Meier Graph for % Predicted FVC decline. Numbers are persons at risk at each interval.
Fig. (2)
Fig. (2)
Kaplan-Meier Graph for % Predicted DLco decline. Numbers are persons at risk at each interval.
Fig. (3)
Fig. (3)
Kaplan-Meier Graph of combined endpoint. Numbers are persons at risk at each interval.
See this image and copyright information in PMC

References

    1. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66(7):940–4. - PMC - PubMed
    1. Steen VD, Conte C, Owens GR, Medsger TA Jr. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum. 1994;37(9):1283–9. - PubMed
    1. Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354(25):2655–66. - PubMed
    1. Tashkin DP, Elashoff R, Clements PJ, et al. Effects of 1-year Treatment with Cyclophosphamide on Outcomes at 2 Years in Scleroderma Lung Disease. Am J Respir Crit Care Med. 2007 - PMC - PubMed
    1. Hoyles RK, Ellis RW, Wellsbury J, et al. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum. 2006;54(12):3962–70. - PubMed

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