IntroductionIdiopathic Pulmonary Fibrosis (IPF) is a uniformly fatal progressive fibrotic lung disease with a mean survival of 3-5 years; currently lung transplant is the only therapy that prolongs survival [1,2]. Other Interstitial Lung Diseases (ILDs) have a better prognosis in general, but some progress to advanced fibrotic lung disease that is fatal without Lung Transplantation (LT) [3][4][5][6]. In contrast to other solid organ transplant, lung transplantation volumes continue to grow. Over 3,600 adult lung transplant procedures were reported in 2013 according to the International Society of Heart and Lung Transplantation registry, the highest in a single year to date. ILD/IPF is a leading indication for LT, accounting for 29% of all lung transplants worldwide [7,8], and is the most common diagnosis for LT (37%) in the United States [9]. In general, short-and long-term survival post-LT have continued to improve, but the median survival remains quite low at 5.6 years for all diseases, and lower, 4.5 years, for IPF/ILD [7,8]. The one-year survival of all adults who underwent primary lung transplant between January 1990 and June 2013 was 80%, and improved overtime to 84% in the 2009 to 2013 era. However, the one-year survival for IPF remains significantly lower at 73-76% [7][8][9].Individual ILD/IPF patients demonstrate widely variable clinical courses and survival, with some progressing rapidly to death, and others enjoying years of little or no progression [10,11]. Hence, predicting prognosis and timing of referral for LT is imperative for best patient outcome.Historically, individual variables have been associated with mortality in IPF, but none have predicted prognosis in isolation [12][13][14][15], and other clinical prognostic models combining variables have had little impact [16][17][18] due to difficulty in clinical use and lack of validation. The GAP model was designed to develop a multidimensional prognostic staging system initially for IPF, then for other ILDs, by using commonly measured variables, specifically gender (G), age (A), and pulmonary physiology (P) including Forced Vital Capacity (FVC) and Diffusion Limitation of Carbon monoxide (DLCO) ( Table 1). These models performed well in two separate derivation and validation cohorts with c-index of approximately 70 in the GAP-IPF study and c-index of 74 in the modified GAP-ILD study in predicting mortality at 1, 2, and 3 years [6,19].In response to a flawed time-based waiting list, the Lung Allocation Subcommittee was formed by the Organ Procurement and . Several models for predicting mortality risk have been developed including the GAP (gender, age, physiology) model. Similarly, the Lung Allocation Score (LAS) has been shown to predict the risk of mortality within the first year posttransplant. We hypothesized that a disease specific mortality model (GAP) might be superior compared to the predictive power of the LAS model for overall mortality within the first year posttransplant in patients with IPF and ILD.
MethodsA retrospective analysis of 7...