Rationale: The survival benefit of lung transplantation (LT) in adult patients with cystic fibrosis (CF) is debated. Objectives: We sought to assess the survival benefit of LT in adult patients with CF. Methods: We used data from the United Network for Organ Sharing Registry to identify adult patients with CF on a wait list for LT in the UnitedStatesbetween2005and2009. Survival timeswhileonthewait list andafter LTweremodeledbyuseofaCoxmodel that incorporated transplantation status as a time-dependent covariate. Evolution in lung allocation score (LAS) while on the wait list was used as a surrogate for disease severity.Wefittedamodel for the joint distributionof survival and longitudinal disease process (LAS over time). Measurements and Main Results: A total of 704 adult patients with CF were registered on a wait list during the study period. The cumulative incidence of LT was 39.3% (95% confidence interval, 35.6- 42.9%) at 3 months and 64.7% (61.0-68.4%) at 12 months, whereas the incidence of death while on the wait list at the same times was 8.5% (6.4-10.6%) and 12.9% (10.3-15.5%), respectively. Survival after LT was 96.5% (94.7-98.2%) at 3 months; 88.4% (85.1- 91.8%) at 12 months; and 67.8% (59.9-76.8%) at 3 years. LT conferred a 69% reduction in the instantaneous risk of death (51-80%). The interaction between LAS and LT was significant: the higher the LAS, the greater the survival benefit of LT (P < 0.001). Conclusions: LT confers a survival benefit for adult patients with CF. Copyright

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doi.org/10.1164/rccm.201303-0429OC, hdl.handle.net/1765/41201
American Journal of Respiratory and Critical Care Medicine
Erasmus MC: University Medical Center Rotterdam