Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature.

Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature.
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DOI:
10.1186/1471-2466-14-139
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发表时间:
2014-08-16
影响因子:
3.1
通讯作者:
Esser D
Esser D
中科院分区:
医学3区
文献类型:
--
作者:
Kistler KD;Nalysnyk L;Rotella P;Esser D

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特发性肺纤维化(IPF)是一种病因不明、预后不良的间质性肺炎。目前的药物治疗是有限的,对于合适的患者,肺移植是一个可行的选择。本综述的目的是全面总结IPF患者的肺移植存活率、单肺移植(SLT)与双肺移植(BLT)之间的差异、肺分配评分(LAS),并总结等待名单上的存活率。对1990年至2013年发表在Medline或Embase上的英语研究进行了系统的审查。符合条件的研究是那些报告肺移植后存活或在IPF患者等待名单上的观察性设计的研究。IPF患者移植后的中位生存期估计为4.5年。根据ISHLT和OPTN的资料,一年生存率在75%-81%之间;3年:59%-%;5年:47%-53%。与其他潜在的移植前诊断相比,IPF的移植后存活率较低。在过去的15年里,接受BLT治疗的IPF患者的比例稳步上升。未经调整的分析表明,与SLT相比,BLT的长期存活率有所提高;在对患者特征进行调整后,差异往往会消失。IPF患者在等待名单上的患者中所占比例最大,虽然等待名单时间减少了,但IPF患者的移植数量随着时间的推移而增加。OPTN数据显示,与其他诊断相比,IPF患者的等待名单死亡率更高。在等待移植期间死亡的IPF患者的比例从14%到67%不等。虽然移植后一年的存活率增加,但实施LAS前后并没有显著差异;然而LAS高与低LAS与一年存活率降低有关。IPF在等待肺移植的患者中所占比例最大,与其他诊断相比,IPF与更高的等待名单和移植后死亡率有关。与SLT相比,BLT存活率的提高可能是选择偏见的结果。除了LAS升高的IPF患者与LAS治疗前相比存活率较低的IPF患者外,LAS治疗前后的存活率似乎是相似的。有关移植后发病率结果的数据很少。
Idiopathic pulmonary fibrosis (IPF) is a distinct form of interstitial pneumonia with unknown origin and poor prognosis. Current pharmacologic treatments are limited and lung transplantation is a viable option for appropriate patients. The aim of this review was to summarize lung transplantation survival in IPF patients overall, between single (SLT) vs. bilateral lung transplantation (BLT), pre- and post Lung Allocation Score (LAS), and summarize wait-list survival. A systematic review of English-language studies published in Medline or Embase between 1990 and 2013 was performed. Eligible studies were those of observational design reporting survival post-lung transplantation or while on the wait list among IPF patients. Median survival post-transplantation among IPF patients is estimated at 4.5 years. From ISHLT and OPTN data, one year survival ranged from 75% - 81%; 3-year: 59% - 64%; and 5-year: 47% - 53%. Post-transplant survival is lower for IPF vs. other underlying pre-transplant diagnoses. The proportion of IPF patients receiving BLT has steadily increased over the last decade and a half. Unadjusted analyses suggest improved long-term survival for BLT vs. SLT; after adjustment for patient characteristics, the differences tend to disappear. IPF patients account for the largest proportion of patients on the wait list and while wait list time has decreased, the number of transplants for IPF patients has increased over time. OPTN data show that wait list mortality is higher for IPF patients vs. other diagnoses. The proportion of IPF patients who died while awaiting transplantation ranged from 14% to 67%. While later transplant year was associated with increased survival, no significant differences were noted pre vs. post LAS implementation; however a high LAS vs low LAS was associated with decreased one-year survival. IPF accounts for the largest proportion of patients awaiting lung transplants, and IPF is associated with higher wait-list and post-transplant mortality vs. other diagnoses. Improved BLT vs. SLT survival may be the result of selection bias. Survival pre- vs. post LAS appears to be similar except for IPF patients with high LAS, who have lower survival compared to pre-LAS. Data on post-transplant morbidity outcomes are sparse.
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