肺泡蛋白沉积症胸部高分辨CT与病理改变对比分析

Comparative analysis of chest high resolution CT and pathogenesis in patients with pulmonary alveolar proteinosis

目的 通过分析肺泡蛋白沉积症 (PAP)的胸部高分辨CT(HRCT)表现与相应的病理改变 ,探讨HRCT对PAP的诊断价值。方法 回顾性分析近 3年确诊的 6例PAP患者胸部HRCT与相应病理学改变。结果 6例患者在不同层面可见磨玻璃影 ,其中 2例可见小结节影 ;3例表现为两肺斑片状磨玻璃影与周围肺组织分界清楚 ,呈地图样改变 ;3例因磨玻璃影与小叶间隔增厚交织成铺路石样改变 ;3例可见肺泡实变融合成密度较高的斑片状阴影 ,1例在肺泡实变区可见“空气支气管征”。 6例患者活检肺组织在光镜下显示肺泡腔内充满大量块状或颗粒状嗜伊红物质 ,PAS染色阳性 ,AB染色阴性 ,其中 3例肺泡间隔正常 ,3例肺泡间隔增宽 ,可见慢性炎性细胞浸润、成纤维细胞增生和胶原沉积。结论 PAP典型的胸部HRCT表现与病理改变一致 ,具有一定的特征性 。

Objective The features of pulmonary alveolar proteinosis (PAP) on high resolution computed tomography (HRCT) and in pathological study were compared in order to improve the early diagnosis of this disease.Methods Retrospective analysis of radiographic, pathologic and clinical data of 6 patients with PAP was made.Results 6 patients with PAP presented with ground-glass opacification of the bilateral lungs on HRCT.Typical chest HRCT scan showed “map-like” lesions in 3 cases and “cobble stone-like” lesions in 3 cases.Multifocal consolidative opacities in the lungs were seen on HRCT in 3 cases.Lung biopsies from 6 patients with PAP revealed a prominent positive periodic acid-Schiff intra-alveolar exudates.There were normal alveolar spetasis in 3 patients while interstitial thickening,fibroblast hyperplasia,interstitial fibrosis in the others.Conclusion The characteristic findings of PAP on chest HRCT fairly correspond with pathological changes.