Outcomes of Lung Transplantation in Autoimmune Diseases

Autoimmune diseases are chronic, complex, multisystem diseases. The pulmonary parenchyma and pulmonary vasculature are frequent targets of many autoimmune diseases, including polymyositis, dermatomyositis, systemic sclerosis, systemic lupus erythematosus, mixed connective tissue disease, Sjogren's syndrome, and rheumatoid arthritis. Adults with these diseases are at risk for developing interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). Although therapies for ILD and PAH have advanced over recent years, they remain limited in their ability to control disease progression. Therefore, many adults with autoimmune disease related ILD or PAH progress to end-stage lung disease requiring lung transplantation. Although lung transplantation can be life-saving, many transplant programs are hesitant to offer lung transplantation to those with autoimmune diseases due to concerns about extra-pulmonary involvement that might affect short- and long-term survival. However, outcomes data in lung transplantation candidates with autoimmune diseases are extremely limited, and even absent for some diseases. The overall objectives of this project are to evaluate mortality rates in adults with autoimmune disease who have undergone lung transplantation in the United States since implementation of the lung allocation score system in 2005, and to identify predictors of poor outcome following lung transplantation in adults with autoimmune diseases. The rationale that underlies this proposed research is that identifying and addressing modifiable risk factors in adults with autoimmune diseases before and after lung transplantation will improve their survival.