Pretransplant Considerations in Patients with Pulmonary Fibrosis

Abstract

Lung transplantation is an established treatment modality for patients with end-stage lung disease and can improve both quality of life (QOL) and survival. Over 6400 lung transplants have been performed since the first successful operation in the early 1980s. According to the International Society for Heart and Lung Transplantation Registry, between January 1995 and June 2015, there were 24.5% lung transplants performed worldwide for interstitial lung disease (ILD) idiopathic interstitial pneumonia (IIP), 5.2% for ILD non-IIP, and 36.4% for chronic obstructive pulmonary disease (COPD). Median survival after lung transplantation is currently 5.5 years, and long-term outcomes are limited mainly by primary graft dysfunction, infection, and allograft rejection. When considering candidates for transplantation, the expected posttransplant survival is carefully weighed against the prognosis of the underlying disease and expected survival if transplant is not performed. The aim of lung transplantation is to improve survival and quality of life, and these goals can be achieved for selected patients with idiopathic pulmonary fibrosis and pulmonary fibrosis associated with connective tissue disease. This chapter will summarize the clinical features of the interstitial lung diseases, such as IPF, connective tissue diseases, systemic sclerosis, rheumatoid arthritis, sarcoidosis, and other rare interstitial lung diseases. Comorbid conditions associated with the ILDs will be discussed, and guidelines for lung transplant candidacy and referral will be presented.