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Recent News and Articles on the Keywords: lung disease + scleroderma + disease  Related to the article below (Last Update: 8/5/2008)

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Cyclophosphamide versus Placebo in Scleroderma Lung Disease -
DP Tashkin, R Elashoff, PJ Clements, J Goldin, MD … - New England Journal of Medicine, 2006 - content.nejm.org
... Occult connective tissue diseases mimicking idiopathic interstitial pneumonias ...
Bronchoalveolar Cellular Analysis in Scleroderma Lung Disease: Does Sutton's Law ...

Evaluation and management of scleroderma lung disease using bronchoalveolar lavage.
RM Silver, KS Miller, MB Kinsella, EA Smith, SI … - Am J Med, 1990 - ncbi.nlm.nih.gov
Click here to read Evaluation and management of scleroderma lung disease using
bronchoalveolar lavage. Silver RM, Miller KS, Kinsella MB, Smith EA, Schabel SI. ...

… endothelin type A and B receptor expression in scleroderma-associated fibrotic lung disease. -
DJ Abraham, R Vancheeswaran, MR Dashwood, VS … - The American Journal of Pathology, 1997 - pubmedcentral.nih.gov
... type A and B receptor expression in scleroderma-associated fibrotic lung disease. ...
Unit of Rheumatology and Connective Tissue Diseases, Royal Free ...

… prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease … -
RM Silver, JH Warrick, MB Kinsella, LS Staudt, MH … - J Rheumatol, 1993 - ncbi.nlm.nih.gov
1993 May;20(5):838-44. Cyclophosphamide and low-dose prednisone therapy in patients
with systemic sclerosis (scleroderma) with interstitial lung disease. ...

High resolution computed tomography in early scleroderma lung disease. -
JH Warrick, M Bhalla, SI Schabel, RM Silver - J Rheumatol, 1910 - ncbi.nlm.nih.gov
J Rheumatol. 1991 Oct;18(10):1520-8. High resolution computed tomography in early
scleroderma lung disease. Warrick JH, Bhalla M, Schabel SI, Silver RM. ...

… for Pulmonary Hypertension Due to the Scleroderma Spectrum of Disease: A Randomized, Controlled … -
DB Badesch, VF Tapson, MD McGoon, BH Brundage, LJ … - Annals of Internal Medicine, 2000 - annals.highwire.org
... These multisystem diseases are characterized by connective ... pulmonary hypertension
and diffuse scleroderma more often have interstitial lung disease (13). ...

Bronchoalveolar lavage for evaluation and management of scleroderma disease of the lung -
J Behr, C Vogelmeier, T Beinert, M Meurer, F … - American Journal of Respiratory and Critical Care Medicine, 1996 - Am Thoracic Soc
... Benefit in Patients with Scleroderma and Alveolitis ... of Oxidative Stress in Interstitial
Lung Diseases Am ... Treatment of Diffuse Parenchymal Lung Disease in Adults ...

… and Lung Fibrosis in Murine Sclerodermatous Graft-Versus-Host Disease: A Model for Human Scleroderma … -
LL McCormick, Y Zhang, E Tootell, AC Gilliam - The Journal of Immunology, 1999 - Am Assoc Immnol
... a murine sclerodermatous graft-vs-host disease (Scl GVHD ... that reproduces important
features of scleroderma including skin thickening, lung fibrosis, and up ...

… Cyclophosphamide Pulse Therapy for the Treatment of Lung Disease Associated with Scleroderma -
EM Davas, C Peppas, M Maragou, E Alvanou, D … - Clinical Rheumatology, 1999 - Springer
... Lung Disease Associated with Scleroderma EM ... Evaluation and management of
scleroderma lung disease using bronchoalveolar lavage. Am ...

Severe restrictive lung disease in systemic sclerosis. -
VD Steen, C Conte, GR Owens, TA Medsger Jr - Arthritis Rheum, 1994 - ncbi.nlm.nih.gov
... to identify risk factors for developing severe restrictive lung disease and to ... Using
the University of Pittsburgh Scleroderma Databank, we grouped patients ...

Source: Google Scholar
 
 

Cyclophosphamide, effective in lung disease linked to scleroderma

 
 
Cyclophosphamide, a drug that suppresses the immune system and reduces inflammation, has been shown to be effective in treating lung disease associated with scleroderma, a connective tissue disease.
.
The study found that a significantly higher proportion of patients taking 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.

" Until now, there has been no proven therapy for scleroderma, and 60-70% of patients die within 10 years, " said Donald Tashkin, at the David Geffen School of Medicine - University of California, Los Angeles ( UCLA ), one of the authors.

Although previous studies have suggested that Cyclophosphamide may benefit people suffering from scleroderma, this study, known as the Scleroderma Lung Study, is the first large, randomized, controlled trial to study the drug's effectiveness against scleroderma lung disease. The study, sponsored by the National Institutes of Health, was conducted at 13 Centers throughout the United States.

Scleroderma is a progressive disease that leads to the hardening and tightening of the skin and connective tissues.
The disease can affect the blood vessels and internal organs, and about 80% of patients also have a problem with their lungs that leads to scarring ( fibrosis ).
This scarring is caused by inflammation, in which an abnormal number of white blood cells accumulate in the lungs.
The inflammation and scarring make breathing more difficult.
More than half of scleroderma deaths are due to lung-related problems.

About 150,000 Americans have scleroderma. Three to four times more women than men develop scleroderma. While it can develop at any age, it most commonly begins between the ages of 25 to 55, according to the Scleroderma Foundation.
The cause of scleroderma is still unknown.

Cyclophosphamide is sometimes used as a treatment for leukemia, lymphoma and other types of tumors.

The study included 162 patients whose scleroderma had caused significant impairment of their lung function as well as shortness of breath.
Patients had scleroderma for an average of three years before entering the study.
The patients were only admitted to the study if testing showed they had active inflammation in the lung, since these patients were most likely to respond to an anti-inflammatory drug.

The patients were divided into two groups: one half received Cyclophosphamide for a year and the other half received a placebo.
Tashkin noted that the lung function of 5 patients receiving placebo dropped so much during the course of the study that they started receiving Cyclophosphamide instead.

The study found that a significantly higher proportion of patients on Cyclophosphamide showed improvement in lung function compared with those taking the placebo.
Patients taking the drug also had significantly more improvement and significantly less worsening of breathlessness than those in the placebo group.

Patients taking Cyclophosphamide also reported better functional ability than the placebo group; and they reported having more energy and pep while taking the drug.

The researchers reported more serious adverse events in the Cyclophosphamide group compared with the placebo group, such as five cases of pneumonia in the treatment group compared with one in the placebo group.
The number of deaths in each group was the same-2 patients each. " Nobody died of toxicity from the drug, " Tashkin said. " We know Cyclophosphamide has toxic effects, but you have to balance the efficacy of the drug against its toxicity. It's a desperate disease, and nothing else has proven effective. We feel the benefits outweigh the risks."

Source: International Conference - American Thoracic Society ( ATS ), 2005
 
 
 
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