Researchers at Columbia University College of Physicians and Surgeons in New York reported that adults with interstitial lung disease (ILD) and systemic sclerosis (SSc) undergoing lung transplantation in the United States are almost 50% more likely to die within one year than people who have lung disease, transplantion but no SSc. The article, titled “Survival of Adults with Systemic Sclerosis Following Lung Transplantation: A Nationwide Cohort Study“, appeared in the January 7th issue of Arthtritis & Rheumatology.
SSc is disease of the connective tissue that involves many different bodily systems. It is characterized by diseased blood vessels and fibrosis — the thickening and scarring of connective tissue. ILD refers to a group of diseases that inflame or scar the lungs. Scarring of the lungs is known as pulmonary fibrosis.
Physicians are not always willing to perform lung transplants in people with SSc. The study authors noted: “Many transplant programs are hesitant to offer lung transplantation to those with SSc due to concerns about extra-pulmonary involvement that might affect survival.”
The investigators, led by Elana J. Bernstein of the Division of Rheumatology, Department of Medicine, Columbia University, wanted to determine whether adults with SSc are more likely to die following lung transplantation than people with other types of lung disease.
They studied the already-available medical records of of 229 adults with SSc, 201 with pulmonary arterial hypertenion (but no SSc), and 3,333 with another type of lung disease. The study subjects had a lung transplant in the U.S. between May 4, 2005, and September 14, 2012. The investigators obtained the data from the United Network for Organ Sharing.
Adults with SSc undergoing lung transplantation had a 48% increase in the 1-year rate of death compared to the people with other types of lung diseases. However, there was no difference in the risk of death at 1 year between people with SSc and individuals with a non-SSc-related pulmonary arterial hypertension. Pulmonary arterial hypertension refers to high blood pressure of the lungs. The condition can occur in SSc but also in other lung diseases.
The authors of the study concluded that “A diagnosis of SSc may confer an increased risk of death 1 year following lung transplantation compared to a diagnosis of ILD, but this risk is similar to that of PAH, a widely accepted indication for lung transplantation. Future work should identify modifiable risk factors that can improve transplant outcomes in this population.”
Future studies may focus on how risk of death following lung transplantation can be reduced specifically in people with SSc and non-SSC PAH. Common features between these conditions may provide clues.