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Pulmonary Fibrosis
This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer.
Overview of Pulmonary Fibrosis
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Overview of Treatments
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Cyclophosphamide (Cytoxan)
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Mycophenolate Mofetil (Cellcept)
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Treatments for Pulmonary Fibrosis: Oral and IV Cyclophosphamide (Cytoxan)
Overview of Cyclophosphamide for Pulmonary Fibrosis
Oral Cyclophosphamide (Cytoxan)
IV Cyclophosphamide (Cytoxan)
Overview of Cyclophosphamide for Pulmonary Fibrosis
Cyclophsophamide is given either intravenously (IV) or orally for pulmonary fibrosis. Studies for both treatments have been completed and some studies are still on-going. Each treatment has its advantages and disadvanatges to the patient. As with all treatments for scleroderma, patients respond differently to oral and IV cyclophosphamide treatments.
Cyclophosphamide in systemic sclerosis: still in search of a 'real life' scenario. In systemic sclerosis (SSc), there is no proven treatment to prevent disease progression. In a recent meta-analysis of three randomised controlled trials (RCTs) and six open prospective studies on cyclophosphamide (CYC), no significant changes in lung function were observed. Further RCTs on early SSc are needed to assess the real efficacy of CYC in inducing remission and increasing survival. I. Miniati. Arthritis Research & Therapy 23 January 2009 (Also see: Cytoxan)
Oral Cyclophosphamide (Cytoxan)
Scleroderma Lung Study II. This study compares 2 different medications—daily oral cyclophosphamide (CYC, also called CytoxanTM) with daily oral mycophenolate mofetil (MMF, also called CellceptTM) in the treatment of scleroderma-related pulmonary fibrosis. There are twelve study centers across the U.S. This study is currently recruiting. University of California, Los Angeles. November 2009. (Also see: Pulmonary Fibrosis, Cellcept, and Clinical Trials)
Oral Cyclophosphamide (CYC) for Active Scleroderma Lung Disease (SSc-ILD): A Decision Analysis. CYC therapy for 1 y results in a small loss in quality of life years compared with no CYC for SSc-ILD. The lack of a beneficial impact on survival and the transience of CYC's impact on decline in pulmonary function drive this conclusion. D Khanna. (Sage Journals) Medical Decision Making, Vol. 28, No. 6, 926-937 (2008).
Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and a meta-analysis of randomized controlled trials and observational prospective cohort studies. Cyclophosphamide treatment in patients with systemic sclerosis related interstitial lung disease does not result in clinically significant improvement of pulmonary function. (UnBound) C. Nannini. Arthritis Res Ther 2008 Oct 20; 10(5):R124. (Also see: Cytoxan,and Clinical Trials)
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)
 
Go to Pulmonary Fibrosis Treatments: Lung Transplants
 
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