支气管镜肺活检在肺泡蛋白沉积症中的诊断价值

目的：评价支气管镜肺活检(transbronchial lung biopsy，TBLB)在肺泡蛋白沉积症(pulmonary alveolar proteinosis，PAP)中的诊断价值和限度。方法：对2006年6月至2012年12月在中南大学湘雅二医院住院、资料完整的 TBLB病理确诊和非TBLB诊断的PAP 25例及TBLB病理误诊为PAP 4例进行回顾性分析。结果：25例PAP患者中，14例 一次TBLB病理确诊一次TBLB阳性率为56%(14/25)]，一次TBLB阴性病例中6例再次TBLB确诊二次TBLB阳性率为 24%(6/25)]，总阳性率为80%(20/25)，总阴性率为20%(5/25)。其中，5例经典型CT表现及支气管肺泡灌洗液检查确 诊，并经全肺灌洗证实；而同期TBLB病理检查将其他疾病误诊为PAP者4例。结论：TBLB是很好的诊断方法，但一 次TBLB阴性不能排除PAP，条件允许可重复进行；临床和CT表现典型结合肺泡灌洗液检查阳性可作为临床诊断依 据；不典型的病例需进行TBLB。

Diagnostic value of transbronchial lung biopsy in pulmonary alveolar proteinosis

Objective: To evaluate the diagnostic value and limits of transbronchial lung biopsy (TBLB) in pulmonary alveolar proteinosis (PAP). Methods: The complete hospital data from Second Xiangya Hospital, Central South University, between June, 2006 and December, 2012, were analyzed retrospectively in 25 patients with PAP (who were diagnosed pathologically by TBLB or not by TBLB) and in 4 patients with other disease (who were misdiagnosed by TBLB). Results: Among the 25 patients with PAP, 14 patients were confi rmed by TBLB in the fi rst time TBLB positive rate in the first time was 56% (14/25)]; 6 patients who were misdiagnosed by TBLB in the fi rst time were confi rmed by TBLB in the second time the positive rate in the second time was 24% (6/25)]. Th e total positive rate was 80% (20/25). Th e total negative rate was 20% (5/25). Five patients with PAP, who showed negative results in TBLB analysis, were confirmed by the typical CT as well as the whole lung lavage. In addition, 4 patients with other diseases were misdiagnosed as PAP by TBLB. Conclusion: TBLB is a very good diagnosis method. But the negative results in the first time cannot exclude PAP. TBLB should be repeated if conditions allow. If clinical manifestation and CT results are typical, it can be used for diagnosis of PAP combined with the positive results from bronchoalveolar lavage fluid check. However, for non-typical cases, it needs TBLB pathological diagnosis.