Dawkins P A, Dowson L J, Guest P J, Stockley R A
Lung Investigation Unit, Queen Elizabeth Hospital, Birmingham, UK.
Thorax. 2003 Dec;58(12):1020-6. doi: 10.1136/thorax.58.12.1020.
Lung density measurements by computed tomography have previously been found to be a more sensitive indicator of disease progression in emphysema of alpha(1)-antitrypsin deficiency than lung function measurements. The aim of this study was to investigate the predictive potential of several parameters, including CT scanning, for mortality in patients with severe alpha(1)-antitrypsin deficiency.
Over a 5 year period, 256 patients with alpha(1)-antitrypsin deficiency (PiZ phenotype) were assessed, of whom 254 underwent lung function testing and 197 had thoracic CT scans. Lung function, CT scans, health status (St George's Respiratory Questionnaire, SGRQ), and other clinical data of survivors and non-survivors were compared and these parameters were applied to survival analyses.
There were 22 deaths in this patient cohort, 10 of which were classified as "respiratory" deaths. Baseline lung function parameters (forced expiratory volume in 1 second (FEV(1)), carbon monoxide transfer coefficient (KCO)), and CT scores were significantly lower in the non-survivors than in the survivors. 170 of the 256 patients had complete data for entry into multiple regression analyses (Cox proportional hazards model). In the univariate analysis, upper zone expiratory scan had the best association with all cause (p = 0.001) and respiratory mortality (p<0.001), whereas FEV(1) (p = 0.158 all cause, 0.015 respiratory) and KCO (p = 0.002 all cause, 0.012 respiratory) had poorer associations with mortality. Only age gave further independent predictive information regarding all cause or respiratory mortality when the CT scan was entered into the survival analyses.
CT scanning predicts respiratory and all cause mortality in alpha(1)-antitrypsin deficiency and appears to be superior to lung function parameters, especially FEV(1).
既往研究发现，对于α1-抗胰蛋白酶缺乏所致肺气肿，通过计算机断层扫描测量肺密度是比肺功能测量更敏感的疾病进展指标。本研究旨在调查包括CT扫描在内的多个参数对重度α1-抗胰蛋白酶缺乏患者死亡率的预测潜力。
在5年期间，对256例α1-抗胰蛋白酶缺乏（PiZ表型）患者进行评估，其中254例接受了肺功能测试，197例进行了胸部CT扫描。对幸存者和非幸存者的肺功能、CT扫描、健康状况（圣乔治呼吸问卷，SGRQ）及其他临床数据进行比较，并将这些参数应用于生存分析。
该患者队列中有22例死亡，其中10例被归类为“呼吸性”死亡。非幸存者的基线肺功能参数（第1秒用力呼气量（FEV1）、一氧化碳转运系数（KCO））和CT评分显著低于幸存者。256例患者中有170例具有完整数据可纳入多元回归分析（Cox比例风险模型）。在单因素分析中，上叶呼气扫描与全因死亡率（p = 0.001）和呼吸性死亡率（p<0.001）的相关性最佳，而FEV1（全因死亡率p = 0.158，呼吸性死亡率p = 0.015）和KCO（全因死亡率p = 0.002，呼吸性死亡率p = 0.012）与死亡率的相关性较差。当将CT扫描纳入生存分析时，只有年龄提供了关于全因或呼吸性死亡率的进一步独立预测信息。
CT扫描可预测α1-抗胰蛋白酶缺乏患者的呼吸性和全因死亡率，且似乎优于肺功能参数，尤其是FEV1。
