摘要
目的探讨肺泡蛋白沉积症(PAP)的临床特点和MSCT表现,分析其特征,以提高诊断准确率。方法回顾性分析19例经支气管肺泡灌洗液(BALF)或经支气管镜肺活检(TBLB)病理证实的PAP患者的临床资料和CT表现。结果 PAP主要临床症状为活动后气促(57.9%)及咳嗽、咳痰(42.1%),12例(63.2%)患者存在不同程度低氧血症或呼吸衰竭,11例(57.9%)患者肺功能表现为不同程度的限制性通气功能障碍伴弥散功能减低。PAP在CT上以"地图样分布"及双肺弥漫分布最为常见(分别约78.9%、63.2%)。在CT征象上,磨玻璃影(100%)及"铺路石征"(84.2%)有一定的特征,此外PAP还可表现为实变(31.6%)、蜂窝肺(10.5%)、牵拉支扩(21.1%)、囊腔及肺大泡(26.3%)等。结论 PAP的临床表现缺乏特异性,CT表现有一定的特征性,且能准确反映病变的范围及严重程度,但确诊仍依赖BALF和TBLB,全肺灌洗是最主要的治疗方式。
Objective To describe the clinical and MSCT features of pulmonary alveolar proteinosis（PAP） and analyze its characteristics and improve the diagnostic accuracy. Methods A retrospective analysis of clinical data and CT findings from 19 patients with pathologically proven PAP by bronchoalVeolar lavage fluid （BALF） or transbronchial lung biopsy（TBLB） was performed. Results Symptoms of PAP included shortness of breath after the activity （57.9%）, cough and sputum （42.1%）, 12 cases （63.2%） had different degrees of hypoxemia and respiratory failure, 11 cases （57.9%） of lung function showed different degrees of restrictive lung function and reduced diffusion capacity. The dominant pattern were geographic distribution and diffuse distribution （78.9%,63.2%） on CT. The CT signs of ground-glass opacity and crazy-paving pattern are common and specific. Consolidation（31.6%）, honeycombing（10.5%）, the traction bronchiectasis（21.1%）, cysts and bullae（26.3%） occurred less frequently. Conclusion The clinical manifestations of PAP lack of specificity. The CT findings are characteristic and can reflect the range and extent of the lesion. The final diagnosis rely on cytological analysis of BALF or TBLB. The main treatment for PAP is the whole-lung lavage.
作者
李菲
唐笑先
师建强
杜晓兢
Li Fei Tang Xiaoxian Shi Jianqiang Du Xiaojing.(Department of Computed Tomography, Shanxi Province People＇s Hospital, Taiyuan 030012, China)
出处
《实用医学影像杂志》
2017年第5期406-409,共4页
Journal of Practical Medical Imaging
关键词
肺泡蛋白沉积症
临床特点
体层摄影术
螺旋计算机
Pulmonary alveolar proteinosis
Clinical characteristics
Tomography, spiral computed
