POSSIBLE NEW ROLE FOR LUNG TRANSPLANTATION IN SCLERODERMA

Reproduced by kind permission of the Scleroderma Foundation of Victoria Incorporated.

The role of lung transplantation for those with scleroderma has long been a point of contention in the medical community but that might change, according to research presented at the American College of Rheumatology Annual Scientific Meeting 2003 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. The investigators found a similar overall survival following transplantation for those with scleroderma compared with idiopathic pulmonary fibrosis and primary pulmonary hypertension; the latter conditions represent two well-established reasons for lung transplantation. There was, however, a trend towards a decrease in survival within the first six months after transplantation for those with scleroderma.

For those unfamiliar with the condition, scleroderma is a chronic autoimmune disorder of the connective tissue characterised by abnormalities in the skin, blood vessels, and internal organs. The term scleroderma means "hard skin" which describes thickening of the skin from increased deposits of collagen. The effects of scleroderma on an individual depend upon the location and extent of collagen deposition. The most common causes of death related to scleroderma are pulmonary fibrosis and pulmonary hypertension.

"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 Dr. Lionel Schachna, formerly a Postdoctoral Fellow at 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". After completing a postdoctoral fellowship in rheumatology and Ph.D. in clinical investigation at Johns Hopkins, Dr. Schachna was recently appointed as Consultant Rheumatologist at the Austin & Repatriation Medical Centre in Melbourne.

The American College of Rheumatology (ACR) is the professional organisation 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 www.rheumatology.org)