Lung Transplants Might be Effective for Scleroderma

Newswise — The role of lung transplantation for people who suffer from scleroderma, an unusual condition that affects 300,000 Americans, has long been a point of contention in the medical community but that might change, according to research presented this week at the American College of Rheumatology Annual Scientific Meeting in Orlando, Florida.

In the largest such study, investigators at The Johns Hopkins Hospital and University of Pittsburgh Medical Center compared survival following lung transplantation of 29 individuals with scleroderma with 70 patients with idiopathic pulmonary fibrosis (scarring of the lung without a known cause) and 38 patients with primary pulmonary hypertension (increased pressure of the circulation to the lungs without a known cause). These two conditions were chosen as comparison groups because they are the principal reasons for lung transplantation in scleroderma. Although there was a trend towards a decrease in survival within the first six months after transplantation for those with scleroderma, the investigators found a similar overall survival for those with scleroderma compared with idiopathic pulmonary fibrosis and primary pulmonary hypertension, which represent two well-established reasons for lung transplantation, suggesting that the long term outcome of patients with this severe disease complication who undergo transplantation warrants the procedure.

Scleroderma is a chronic autoimmune disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs. The term scleroderma means "hard skin," which describes thickening of the skin from increased deposits of collagen. There are two types of scleroderma. Localized scleroderma affects the skin in limited areas and the musculoskeletal system. Systemic sclerosis causes more widespread skin changes and may be associated with internal organ damage in the lungs, heart and kidneys.

"Our findings indicate that in an appropriately screened individual, the diagnosis of scleroderma, in and of itself, should not preclude the consideration of a lung transplant" , said Lionel Schachna, MD, PhD, Postdoctoral Fellow, Johns Hopkins University, Baltimore, Maryland, and lead investigator of the study. "Efforts should now be focused on those first few months following transplantation in order to even further improve the outcome for our scleroderma patients."

The American College of Rheumatology is the professional organization for rheumatologists and health professionals who share a dedication to healing, preventing disability and curing arthritis and related rheumatic and musculoskeletal diseases. For more information on the ACR's annual meeting, see http://www.rheumatology.org/annual.