Lung Transplantation for Connective Tissue Disorders

Connective tissue disorders represent a group of systemic autoimmune collagen vascular diseases that often lead to life-threatening end-stage interstitial lung disease and severe secondary pulmonary hypertension, needing lung or combined heart-lung transplantation as a treatment option. Controversies remain whether lung transplantation is a justifiable therapeutic option in patients with connective tissue disorders, and often lung transplant centers do not consider these patients as acceptable candidates. Concerns regarding surgical challenges and underlying medical complexities, particularly esophageal dysmotility and gastroesophageal reflux disease with risk for chronic allograft dysfunction, have contributed to most centers’ declining to perform lung transplants for patients with underlying connective tissue lung disease. Current literature from the United States and Europe on outcomes of lung transplantation in patients with connective tissue disorders showed a comparable survival to other patients with interstitial lung disease nonrelated to connective tissue disorders, indicating that lung transplantation is a reasonable treatment option for selected patients with connected tissue disorders.