肺泡蛋白沉着症X线胸片及高分辨率CT表现与肺功能的关系

X-ray and high resolution CT findings in pulmonary alveolar proteinosis and their relationship with pulmonary function

目的探讨肺泡蛋白沉着症X线胸片及胸部高分辨率CT（HRCT）影像表现及其与肺功能检查的相关性。方法回顾性分析19例肺泡蛋白沉着症患者的X线胸片、胸部HRCT及肺功能检查[肺总量、一氧化碳弥散量（DLCO）、第一秒用力肺活量（FEV1）及一秒率（FEV1／FVC）]，根据肺部阴影的程度、范围、严重程度及网格阴影范围进行影像学评分，将X线胸片及HRCT的影像评分与肺功能指标进行相关性分析。结果X线胸片以双侧对称的自肺门向外放射的“蝶翼征”表现为主，胸部HRCT特征性的表现为两肺弥漫性分布的斑片状磨玻璃样气腔实变影，伴小叶间隔增厚及网格影，这种“铺路石”样病变呈地图样分布。19例患者的肺功能结果为DLCO占预计值百分比：（54．49±16．78）％；FEV1／FVC：（86．00±6．70）％。X线胸片的范围评分及严重度评分与DLCO占预计值百分比呈负相关（r=-0．661，P〈0．01；r=-0．475，P〈0．05）。HRCT严重度评分为（16．47±5．26）分，在HRCT的各项评分中严重度评分与限制性通气功能障碍（FEV1／FVC升高）及弥散功能障碍（DLCO占预计值百分比下降）的相关性最好（DLCO占预计值百分比r=-0．809，P〈0．01；FEV，／FVCr=0．573，P〈0．05）；HRCT的范围评分比程度评分与肺功能的相关性更好，而HRCT的网格范围评分只与弥散功能障碍相关。结论肺泡蛋白沉着症x线胸片、胸部HRCT与肺功能相关性良好，HRCT比X线胸片更能准确反映肺功能受损情况。

Objective To study X-ray and high resolution computed tomograghy （HRCT） features and their relationship with pulmonary function test in pulmonary alveolar proteinosis（PAP）. Methods The findings of X-ray and HRCT and the results of pulmonary function tests in 19 patients with PAP were reviewed retrospectively. X-ray and HRCT scores were rated in the aspects of opacity density, extent, and severity. The relationship between the imaging scores and pulmonary function tests were determined. Results X-ray showed a symmetrical perihilar predominance appearance in patients with PAP. HRCT scan showed bilateral clear-defined pathchy areas of ground-glass opacity and interlobular septal thickening, a pattern commonly characterized as ＂crazy paving＂. Nineteen PAP respiratory function results were： DLCO/Pred = （54. 49 ± 16. 78） %, FEV1/FVC = （ 86. 00 ± 6. 70 ） %. Chest radiographic extent score and severity score correlated with diffusing capacity （ r = - 0. 661, P 〈 0. 01, r = - 0. 475, P 〈 0. 05 ）. HRCT severity score was 16.47 ± 5.26. Among all the HRCT scores, severity score correlated significantly with the presence of restrictive ventilatory defect and diffusing capacity defect （ DLCO/Pred, r = - 0. 809, P 〈 0. 01 ; FEV1/FVC, r = 0. 573, P 〈 0. 05 ）. Conclusions In patients with PAP, there is close relationship between X-ray, HRCT findings and pulmonary function. HRCT correlates more closely with pulmonary function.