肺泡蛋白沉着症早期诊断体会

THE EARLY DIAGNOSIS OF PULMONARY ALVEOLAR PROTEINOSIS

目的对9例确诊肺泡蛋白沉着症(PAP)病人进行分析,探讨PAP的早期诊断体会。方法对9例经纤维支气管镜肺泡灌洗液(BALF)病理检查证实的PAP病人进行回顾性分析,仔细观察BALF治疗前后PAP病人临床表现及影像学特征性变化。结果9例病人均有进行性加重的活动性呼吸困难,高分辨率CT检查示双肺弥漫分布毛玻璃样淡薄影,有地图样表现,小叶间隔增厚,呈斑片状影,有碎石路样表现。结论进行性加重的活动性呼吸困难、慢性呼吸衰竭、比较特征性影像学表现与BALF的PAS阳性染色是早期诊断本病的关键。

Objective To study the early diagnosis of pulmonary alveolar proteinosis （PAP） by analyzing the data of patients with confirmed PAP. Methods A retrospective analysis was done in nine patients with PAP who were identified through bronchoalveolar lavage fluid （BALF） pathologically. Their clinical symptoms and radiographic features were reviewed, before and after BALF therapy. Results All patients showed progressive dyspnea and radiography showed bilateral shadows, thickening of interlobular septum. Conclusion The key of the early diagnosis of this condition is based on combination of the symptoms, imageological features and BALF with positive PAS.