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特发性肺纤维化合并肺气肿时的功能和预后影响。

Functional and prognostic effects when emphysema complicates idiopathic pulmonary fibrosis.

机构信息

Department of Radiology, Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust, London, UK

Division of Radiology, Mayo Clinic Rochester, Rochester, Minnesota, USA.

出版信息

Eur Respir J. 2017 Jul 5;50(1). doi: 10.1183/13993003.00379-2017. Print 2017 Jul.

Abstract

This study aimed to investigate whether the combination of fibrosis and emphysema has a greater effect than the sum of its parts on functional indices and outcome in idiopathic pulmonary fibrosis (IPF), using visual and computer-based (CALIPER) computed tomography (CT) analysis.Consecutive patients (n=272) with a multidisciplinary IPF diagnosis had the extent of interstitial lung disease (ILD) scored visually and by CALIPER. Visually scored emphysema was subcategorised as isolated or mixed with fibrotic lung. The CT scores were evaluated against functional indices forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide (), transfer coefficient of the lung for carbon monoxide (), composite physiologic index (CPI)) and mortality.The presence and extent of emphysema had no impact on survival. Results were maintained following correction for age, gender, smoking status and baseline severity using , and combined visual emphysema and ILD extent. Visual emphysema quantitation indicated that relative preservation of lung volumes (FVC) resulted from tractionally dilated airways within fibrotic lung, ventilating areas of admixed emphysema (p<0.0001), with no independent effect on FVC from isolated emphysema. Conversely, only isolated emphysema (p<0.0001) reduced gas transfer ().There is no prognostic impact of emphysema in IPF, beyond that explained by the additive extents of both fibrosis and emphysema. With respect to the location of pulmonary fibrosis, emphysema distribution determines the functional effects of emphysema.

摘要

本研究旨在通过视觉和基于计算机的(CALIPER)计算机断层扫描(CT)分析,探讨特发性肺纤维化(IPF)中纤维化和肺气肿的联合是否比其各部分的总和对功能指标和预后有更大的影响。

对具有多学科 IPF 诊断的连续患者(n=272)进行了视觉和 CALIPER 评估的间质性肺病(ILD)程度评分。视觉评分的肺气肿分为孤立型或与纤维化肺混合。对 CT 评分进行了评估,评估指标为用力肺活量(FVC)、一氧化碳弥散量()、一氧化碳肺转移系数()、综合生理指数(CPI)和死亡率。

肺气肿的存在和程度对生存率没有影响。使用校正年龄、性别、吸烟状况和基线严重程度后,结果得到了维持,同时校正了视觉肺气肿和ILD 程度。视觉肺气肿定量分析表明,纤维化肺中牵拉性扩张的气道导致相对保留肺容积(FVC),通气混合性肺气肿区域(p<0.0001),孤立性肺气肿对 FVC 无独立影响。相反,只有孤立性肺气肿(p<0.0001)降低了气体转移()。

在 IPF 中,除了纤维化和肺气肿的程度相加之外,肺气肿没有预后影响。就肺纤维化的位置而言,肺气肿的分布决定了肺气肿的功能影响。

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