肺泡蛋白沉着症的影像学表现及鉴别诊断

Imaging of pulmonary alveolar proteinosis and differential diagnosis

目的:了解肺泡蛋白沉着症(PAP)的临床和影像学表现,加强对PAP的影像学诊断及鉴别诊断的认识,提高PAP的诊断准确率。方法:对经支气管肺泡灌洗(BALF)和纤支镜下肺活检(TBLB)确诊的8例PAP患者的影像学资料及临床表现进行回顾性分析。结果:PAP患者胸部X线表现呈非特异性改变;CT表现多样,为磨玻璃样、碎石路样、地图样、小叶间隔增厚样、肺实变样、肺水肿样及肺间质纤维化等表现,临床表现为咳嗽,活动后气促、胸痛、胸闷、进行性呼吸困难。结论:肺部影像可充分显示PAP的特点,结合临床可提示本病可能,但其特点只具相对特异性,应尽早行TBLB和BALF以确诊。临床上需与卡氏肺囊虫性肺炎、肺水肿、肺出血、支气管肺泡癌、感染、特发性纤维化等肺部疾病进行鉴别诊断。

To explore clinical and imaging manifestation of pulmonary alveolar proteinosis （ PAP ）, and differential diagnosis. Methods：Clinical data of 8 cases with PAP confirmed by bronchoalveolar lavage fluid or transbronchial lung biopsy were analyzed retrospectively. Results. All patients showed nonspecifical change in chest Xray imaging, and various CT manifestation, including ground glass pattern, crazing paving pattern, map pattern, thickening interlobular septum, pulmonary consolidation, pulmonary edema and pulmonary interstitial fibrosis, etc~ while clinical symptoms included cough, shortness of breath after activity, chest pain, chest tightness, progressive dyspnea. Conclusion. The imaging can reveal the characteristic of PAP, but it is relatively specific. It should be diagnosed by bronchoalveolar lavage fluid or transbronchial lune biopsy as soon as possible.