目的探讨肺泡蛋白沉积症（pulmonary alveolar proteinosis，PAP）的临床病理特征。方法分析 2014 年—2018 年四川大学华西医院 25 例经病理确诊为 PAP 患者的临床病理资料。结果PAP 患者 25 例，年龄 18～73 岁，平均（42.52±15.79）岁。PAP 好发于男性患者（20 例）。最常见的类型是自身免疫性 PAP（15 例），继发性 PAP （10 例）见于肺炎、结核、肾病综合征及尘肺患者。PAP 常见临床症状为咳嗽（24 例）、咳痰（20 例）及进行性加重的呼吸困难（气紧气促 11 例 、呼吸困难 5 例）。胸部 CT 表现为双肺磨玻璃影（19 例）、网格样改变（11 例）及地图样改变（3 例）。肺活检（肺泡腔内）和（或）肺泡灌洗液见过碘酸雪夫（periodic acid-schiff，PAS）染色和消化后 PAS 染色双阳性脂蛋白样物沉积。结论PAP 无特征性临床症状，确诊周期长。临床医生应该结合临床症状、影像学特征以及肺活检和（或）肺泡灌洗液 PAS 染色和消化后 PAS 染色以明确诊断。

ObjectiveTo explore the clinicopathological features of pulmonary alveolar proteinosis (PAP).MethodsA total of 25 patients with PAP who were pathologically diagnosed in West China Hospital of Sichuan University from 2014 to 2018 were collected as the study subjects.ResultsThe 25 patients with PAP were 18–73 years old, with an average age of (42.52±15.79) years. There were 20 males and 5 females. The most common type was autoimmune PAP (15 cases), and secondary PAP (10 cases) were found in patients with pneumonia, tuberculosis, nephrotic syndrome, and pneumoconiosis. The common clinical symptoms of PAP were cough (24 cases), expectoration (20 cases), and progressive difficulty in breathing (11 cases of shortness of breath and 5 cases of dyspnea). The chest CT manifestations included double lung grinding (19 cases), grid-like changes (11 cases), and map-like changes (3 cases). Periodic acid-schiff (PAS) staining and post-digestion PAS staining double positive lipoprotein-like deposits was observed in lung biopsy (in the alveolar cavity) and/or alveolar lavage fluids.ConclusionsPAP has no characteristic clinical symptoms and a long diagnosis period. Clinicians should combine clinical signs, imaging features, and lung biopsy and/or alveolar lavage fluid PAS staining and post-digestion PAS staining to confirm the diagnosis.

关键词: 肺泡蛋白沉积症; 肺泡灌洗液; 肺活检

Key words: Pulmonary alveolar proteinosis; Alveolar lavage fluid; Lung biopsy

引用本文： 刘庆林, 蒋莉莉. 肺泡蛋白沉积症 25 例临床病理分析. 华西医学, 2019, 34(1): 18-21. doi: 10.7507/1002-0179.201812076 复制