ABSTRACT
End-stage lung disease due to myriad interstitial lung diseases (ILDs) accounts for approximately 20% of lung transplants (LTs) performed worldwide (1). Data from the International Society of Heart and Lung Transplantation (ISHLT) Registry from January 1995 to June 2004 cited idiopathic pulmonary fibrosis (IPF) as the second most frequent diagnosis leading to LT, comprising 17% of all LTs (1). Sarcoidosis and collagen vascular disease-associated pulmonary fibrosis (CVD-PF) accounted for 2.5% and 0.5% of LTs performed in that time frame, respectively (1). The prognoses of these diverse ILDs differ, and criteria for listing patients with these disorders have not been validated. When to list patients for LT is a difficult decision, since predicting life expectancy in individual patients is imperfect at best. Further, waiting times for organ procurement are unpredictable, but may be prolonged. Unfortunately, death rates while awaiting LT are highest in patients with IPF compared to other disorders. Early listing of
patients with IPF is critical to improve outcome. Given the rarity of ILDs, appropriate timing for listing for LT has not been well established. In this chapter, we first discuss IPF because there is the greatest amount of experience and published data relating to this disorder. We later discuss the role of LT for patients with CVD-PF and sarcoidosis.
