Mortality on the Waiting List for Lung Transplantation in Patients with Idiopathic Pulmonary Fibrosis: A Single-Centre Experience
Mortality on the Waiting List for Lung Transplantation in Patients with Idiopathic Pulmonary Fibrosis: A Single-Centre Experience
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DOI：
10.1007/s00408-015-9767-x
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发表时间：
2015-10-01
期刊：
影响因子：
5
通讯作者：
Rottoli, Paola
中科院分区：
文献类型：
--
作者：
Bennett, David;Fossi, Antonella;Rottoli, Paola
目的 肺移植 (LTX) 目前已被接受为特定终末期肺病患者的治疗选择。特发性肺纤维化（IPF）的特征是放射学和组织学表现与普通间质性肺炎相同。它与不良预后相关，LTX 被认为是一种有效的治疗方法，可以显着改变这种疾病的自然史。本研究的目的是分析单个机构中 IPF 患者等待名单期间的死亡率。方法对 2001-2014 年期间转诊至我们的肺移植项目的 IPF 患者 (n = 90) 进行回顾性分析，重点关注患者的特征和相关危险因素。结果相对于其他诊断，IPF 的诊断与等待名单上的高死亡率相关 (p < 0.05)。在接受 LTX 或不在等待名单上的 IPF 患者之间，没有观察到人口统计学、临床、放射学数据和在等待名单上花费的时间存在差异。死亡患者的 pCO(2) 水平显着升高，并且需要更高流量的 O-2 治疗（p < 0.05）。肺功能测试未能预测死亡率，并且没有其他医疗状况与生存相关。 结论 新诊断为 IPF 的患者，特别是在中小型肺移植中心和预计等待名单很长的国家，应立即转诊至移植，延迟会导致死亡率增加。迫切需要早期识别具有快速进展表型的 IPF 患者。
Purpose Lung transplantation (LTX) is nowadays accepted as a treatment option for selected patients with end-stage pulmonary disease. Idiopathic pulmonary fibrosis (IPF) is characterized by the radiological and histologic appearance of usual interstitial pneumonia. It is associated with a poor prognosis, and LTX is considered an effective treatment to significantly modify the natural history of this disease. The aim of the present study was to analyse mortality during the waiting list in IPF patients at a single institution.Methods A retrospective analysis on IPF patients (n = 90) referred to our Lung Transplant Program in the period 2001-2014 was performed focusing on patients' characteristics and associated risk factors.Results Diagnosis of IPF was associated with high mortality on the waiting list with respect to other diagnosis (p < 0.05). No differences in demographic, clinical, radiological data and time spent on the waiting list were observed between IPF patients who underwent to LTX or lost on the waiting list. Patients who died showed significant higher levels of pCO(2) and needed higher flows of O-2-therapy on effort (p < 0.05). Pulmonary function tests failed to predict mortality and no other medical conditions were associated with survival.Conclusions Patients newly diagnosed with IPF, especially in small to medium lung transplant volume centres and in Countries where a long waiting list is expected, should be immediately referred to transplantation, delay results in increased mortality. Early identification of IPF patients with a rapid progressive phenotype is strongly needed.