Background: Idiopathic pulmonary fibrosis (IPF) is a distinct form of interstitial pneumonia with unknown origin\nand poor prognosis. Current pharmacologic treatments are limited and lung transplantation is a viable option for\nappropriate patients. The aim of this review was to summarize lung transplantation survival in IPF patients overall,\nbetween single (SLT) vs. bilateral lung transplantation (BLT), pre- and post Lung Allocation Score (LAS), and\nsummarize wait-list survival.\nMethods: A systematic review of English-language studies published in Medline or Embase between 1990 and\n2013 was performed. Eligible studies were those of observational design reporting survival post-lung transplantation\nor while on the wait list among IPF patients.\nResults: Median survival post-transplantation among IPF patients is estimated at 4.5 years. From ISHLT and OPTN data,\none year survival ranged from 75% - 81%; 3-year: 59% - 64%; and 5-year: 47% - 53%. Post-transplant survival is lower for\nIPF vs. other underlying pre-transplant diagnoses. The proportion of IPF patients receiving BLT has steadily increased over\nthe last decade and a half. Unadjusted analyses suggest improved long-term survival for BLT vs. SLT; after adjustment for\npatient characteristics, the differences tend to disappear. IPF patients account for the largest proportion of patients on\nthe wait list and while wait list time has decreased, the number of transplants for IPF patients has increased over time.\nOPTN data show that wait list mortality is higher for IPF patients vs. other diagnoses. The proportion of IPF patients who\ndied while awaiting transplantation ranged from 14% to 67%. While later transplant year was associated with increased\nsurvival, no significant differences were noted pre vs. post LAS implementation; however a high LAS vs low LAS was\nassociated with decreased one-year survival.\nConclusions: IPF accounts for the largest proportion of patients awaiting lung transplants, and IPF is associated\nwith higher wait-list and post-transplant mortality vs. other diagnoses. Improved BLT vs. SLT survival may be the\nresult of selection bias. Survival pre- vs. post LAS appears to be similar except for IPF patients with high LAS,\nwho have lower survival compared to pre-LAS. Data on post-transplant morbidity outcomes are sparse.
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