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Pulmonary Fibrosis
This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer.
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Treatments for Pulmonary Fibrosis: Oral Cyclophosphamide (Cytoxan)
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Cyclophosphamide (Cytoxan)
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Oral Cyclophosphamide (Cytoxan)
Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. One year of CYC improved lung function, skin scores, dyspnea, and health status/disability, effects which either persisted or increased further for several months after stopping therapy. However, except for a sustained impact on dyspnea, all of these effects waned and were no longer apparent at 24 months. PubMed. Am J Respir Crit Care Med. 2007 Nov 15;176(10):1026-34. (Also see: Medications and Cytoxan )
Cyclophosphamide versus Placebo in Scleroderma Lung Disease. One year of oral cyclophosphamide for symptomatic scleroderma-related interstitial lung disease had a significant but modest beneficial effect on lung function, dyspnea, thickening of the skin, and the health-related quality of life. The New England Journal of Medicine. Volume 354:2655-2666:25, June 22, 2006. (Also see: Clinical Trials)
Oral cyclophosphamide (CYC) improves pulmonary function in scleroderma patients with fibrosing alveolitis: experience in one centre. Long-term therapy with oral CYC is effective in ameliorating or stabilising lung function in scleroderma patients with active alveolitis, with beneficial effects lasting up to 1 year after interruption. PubMed. Clin Rheumatol. 2006 Apr 14. (Also see: Medications and Cyclophosphamide)
Long Term Pulmonary Function and Survival Outcomes of Scleroderma Patients treated with Cyclophosphamide for active interstitial lung disease. The majority of systemic sclerosis (SSc) patients who are treated with cyclophosphamide for active interstitial lung disease experience long-term lung function stability. However, around 1/3 continue to have lung function decline despite this therapy. Shikha Mittoo. F57/577 ACR 2006. (Also see: Medications and Cyclophosphamide )
Outcomes at 24-months are Better for Patients Who Received One-year of Cyclophosphamide (CYC) Therapy for SSc Alveolitis Than in Those Who Received One-year of Placebo in the Scleroderma Lung Study. Differences between groups with respect to breathlessness, activities of daily living and general well-being were larger and favored CYC. Philip J. Clements. 1252 ACR 2006. (Also see: Clinical Trials: Cyclophosphamide and Scleroderma Lung Disease )
Cyclophosphamide: Study finds drug works against scleroderma lung disease. The study found that a significantly higher proportion of patients taking the drug, cyclophosphamide, showed improvement in lung function compared with those taking a placebo. Patients taking the drug also had significant reductions in breathlessness and reported feeling more energetic and in better health. EurekAlert! 05/23/05.
IV Cyclophosphamide (Cytoxan)
Therapeutic Strategy Combining Intravenous Cyclophosphamide (CYC) Followed by Oral Azathioprine to Treat Worsening Interstitial Lung Disease Associated with Systemic Sclerosis: A Retrospective Multicenter Open-label Study. Intravenous CYC followed by oral maintenance immunosuppressive therapy for worsening ILD was well tolerated and was associated with stable or improved PFT in 70% and 51.8% of SSc patients at 6 months and 2 years, respectively. J Rheumatology First Release May 1 2008. (Also see: Medications )
The Effect Of Cyclophosphamide Treatment On Skin Changes In Patients With Scleroderma Interstitial Lung Disease. Although the lot was small – only 28 patients- because they were treated with Cyclophosphamide according to their lung involvement, the Cyclophosphamide treatment seemed to ameliorate the skin thickening in Systemic Sclerosis. The effect is greater in those patients with higher mRodnanss. I. C. Oprisan. AB0518 EULAR 2007. (Also see: Skin Fibrosis and Cyclophosphamide )
Intermittent intravenous cyclophosphamide pulse therapy for the treatment of active interstitial lung disease associated with collagen vascular diseases. The efficacy and safety of our “divided administration” protocol of CYC for CVD-ILD was demonstrated. IngentaConnect. Modern Rheumatology, Volume 17, Number 2, April 2007, pp. 131-136(6). (Also see: Medications and Cytoxan )
Combination of intravenous pulses of cyclophosphamide and methylprednizolone in patients with systemic sclerosis and interstitial lung disease. The results suggest that the employed combination is safe and effective, mainly in stabilizing the respiratory function of the patients. This goal is more realistic when treatment is given before significant functional compromise has ensued. PubMed. Rheumatol Int. 2007 Feb;27(4):357-61. (Also see: Cyclophosphamide for Scleroderma Lung Disease, and Medications )
Efficacy Evaluation of Cyclophosphamide (CP) for Treating Interstitial lung disease (ILD) in Systemic Sclerosis. The use of iv.CP for treating ILD related to SSc stabilizes the course of this complication and should be started at the beginning of the disease. Evaluation of the efficacy of the treatment must include clinical, pulmonary function and quantified radiological finding criteria. P. García de la Peña Lefebvre. AB0275 EULAR 2006.
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. This suggests that treatment of pulmonary fibrosis in SSc with low-dose prednisolone and IV CYC followed by AZA stabilizes lung function in a subset of patients with the disease. PubMed. Arthritis Rheum. 2006 Dec;54(12):3962-70.
Association of Prolonged Cyclophosphamide Therapy with Pulmonary Function Improvement in Scleroderma. In patients with scleroderma lung, extending the IV CYC therapy beyond one year increases the probability of pulmonary function improvement. G. E. Tzelepisg. OP0123 EULAR 2006.
Cyclophosphamide reduces neutrophilic alveolitis in patients with scleroderma lung disease: a retrospective analysis of serial bronchoalveolar lavage investigations. Previous reports that patients with scleroderma lung disease with increased levels of granulocytes in BALF are more likely to benefit from treatment with intravenous cyclophosphamide are confirmed. PubMed. Ann Rheum Dis. 2005 Sep;64(9):1343-6. (Also see: Medications and Cyclophosphamide)
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