Lung Transplantation in Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease associated with a high degree of morbidity and mortality in its more advanced stages. Antifibrotic therapies are generally effective in delaying the progression of disease; however, some patients continue to progress despite treatment. Lung transplantation is a surgical option for selected patients with advanced pulmonary fibrosis that increases their overall survival and quality of life. Changes in the Lung Allocation Score (LAS) in 2005 have resulted in increased transplants and decreased waitlist mortality in this population. Indications for transplant evaluation and listing include the clinical progression of the disease and related mortality risk ≥50% at 2 years without a transplant. Patients with clinically rapid deterioration or acute flares needing hospitalization can be bridged to transplant on extracorporeal support while remaining ambulatory and free from mechanical ventilation