Cyclophosphamide Improves Breathing for Scleroderma Patients
Jul 10, 2006 - 6:43:37 AM

An oral medication improves overall lung function and reduces lung-related inflammation associated with the deadly skin disease scleroderma, says a researcher at the University of Medicine and Dentistry of New Jersey who was part of a national study.

The 12-month, 13-site clinical trial involved 158 patients treated with the drug cyclophosphamide. The oral medication had a modest but significant effect on the lung functions of scleroderma patients stricken with alveolitis, a chronic inflammation of the lung tissue commonly diagnosed in those with systemic scleroderma, said Dr. Vivien
M. Hsu, a scleroderma specialist at the UMDNJ-Robert Wood Johnson Medical School in New Brunswick and a principal investigator in the study.

Subjects with dyspnea, or breathlessness due to interstitial lung disease, also felt better after being given daily doses of cyclophosphamide for one year, Dr. Hsu said. The study was published recently (June 22, 2006) in the New England Journal of Medicine.

"We used a double-blind, randomized, placebo-controlled clinical trial to determine the drug's ability to suppress labored breathing and restrictive lung physiology in these patients," Dr. Hsu said. "We assessed their pulmonary function every three months to
determine their forced vital capacity regarding breathing, and then closely followed the patients for one year thereafter."

The patients in the study received 2 milligrams per kilogram of body weight each day, or matching placebo, for one year. The forced vital capacity measured in these subjects at the end of two years remained stable in the majority of patients who received cyclophosphamide.

Scleroderma is a rheumatic disease that primarily affects women, and is characterized by skin thickening due to over-production of scar (collagen) and connective tissue. This disease often scars the organs, including the lungs, kidneys, gastro-intestinal tract and heart. Scleroderma frequently can damage the smallest blood vessels leading to
further damage of these vital organs.

The cause of the disease is unknown but it is widely regarded by medical experts as an autoimmune disorder. There are no proven drugs that may reverse the damage due to scleroderma-related interstitial lung disease. Different drugs may be used to treat its manifestations, but patients whose vital organs are attacked and adversely affected by
scleroderma may die from complications of this unpredictable disease.

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