ABSTRACT

I. Introduction Lung transplantation is an effective therapeutic option for a variety of end-stage lung diseases. Chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), cystic fibrosis (CF), and idiopathic pulmonary hypertension (IPAH) are among the more common advanced lung disorders for which lung transplantation is performed. From the 2009 Registry of the International Heart and Lung Transplant Society (ISHLT) report for the period of January 1995 through June 2008 (1), IPF accounts for 21%, or about 5000 lung transplants. Not included in this IPF group is the diagnosis of connective tissue disease related interstitial lung disease (CT-ILD), comprising only 0.8%, or 181 lung replacement procedures. This number has risen from 0.5% as reported from the ISHLT registry of 22nd annual report (2).