Pulmonary function in idiopathic pulmonary fibrosis and referral for lung transplantation

Of patients awaiting lung transplantation, the death rates are highest in t hose with idiopathic pulmonary fibrosis (IPF), suggesting that many IPF pat ients are referred late for transplantation. Therefore this study was under taken to evaluate baseline pulmonary function test (PFT) and high-resolutio n computed tomography (HRCT) fibrosis scores, and the relationship to survi val in IPF patients younger than 65 yr of age. A total of 115 patients with usual interstitial pneumonia (UIP) were studied. At presentation to a tert iary referral center, PFT and HRCT data were collected and analyzed for pro gnostic significance: the primary outcome measure was patient death. Based on the length of the waiting list for transplantation, prediction of 2-yr s urvival was examined. DLCO percent predicted and HRCT-fibrosis score were f ound to be independent predictors of survival and in combination gave the b est prognostic prediction. The optimal points on the receiver operating cha racteristic (ROC) curves for discriminating between survivors and nonsurviv ors corresponded to 39% DLCO percent predicted, and to a HRCT-fibrosis scor e of 2.25. The combination of these parameters yielded an optimal point wit h a specificity and a sensitivity of 84% and 82%, respectively. A model bas ed on a combination of DLCO percent predicted and HRCT-fibrosis score may o ptimize the timing of referral for transplantation.