Clinical characteristics of pulmonary alveolar proteinosis.
Clinical characteristics of pulmonary alveolar proteinosis.
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DOI：
10.11817/j.issn.1672-7347.2021.190792
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发表时间：
2021-02-28
期刊：
影响因子：
--
通讯作者：
Ouyang, Ruoyun
中科院分区：
文献类型：
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作者：
Long, Yingjiao;Liu, Guiqian;Ouyang, Ruoyun
目的：肺泡蛋白沉积症（PAP）是一种少见的疾病，临床表现多样，无特异性，常导致误诊。通过总结PAP的临床特点、诊断和治疗，提高对该病的认识。方法：回顾性分析2008 - 2019年中南大学湘雅二医院呼吸与危重症医学科收治的25例PAP患者的临床资料。不明原因的咳嗽和呼吸困难是PAP常见的临床表现。5例患者有职业性吸入暴露史。16例患者具有典型的影像学特征，包括肺泡腔磨玻璃样影，小叶间隔和小叶内间隔增厚，呈典型的铺路石样和地图样。14例患者进行了肺功能检查，显示一氧化碳弥散能力降低。经支气管镜活检阳性率为95%。5例患者接受全肺灌洗，随访5年后症状和影像学改变明显减轻。经支气管镜肺活检是诊断PAP的有效方法。全肺灌洗仍是一种有效的治疗方法。
OBJECTIVES: Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the awareness of this disease via summarizing the clinical characteristics, diagnosis, and therapy of PAP.METHODS: We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University.RESULTS: Cough with unkown reason and dyspnea were common clinical manifastations of PAP. Five patients had a history of occupational inhalational exposure. Sixteen patients had typical image features including ground-glass opacification of alveolar spaces and thickening of the interlobular and intralobular septa, in typical shapes called crazy-paving and geographic pattern. Fourteen patients underwent pulmonary function tests, revealing a reduction in the diffusing capacity for carbon monoxide. The positive rate of transbronchial biopsy was 95%. Five patients received the whole lung lavage and the symptoms and imaging fcauters significantly relieved after five-years follow-up.CONCLUSIONS: PAP is characterized by radiographic pattern and pathology. Transbronchial lung biopsy is effective to make diagnosis of PAP. The whole lung lavage remains a efficient therapy.