肺泡蛋白沉积症6例临床分析

摘要

Objective:To explore the clinical features, diagnosis and therapy of pulmonary alveolus proteinosis(PAP)and improve the level of clinical diagnosis and treatment to reduce misdiagnosis and incorrect treatment. Methods: Retrospective analysis of the clinical features and chest CT data of patients with PAP in Baoshan People's Hospital in the last 9 years was made, the experience of treatment was summarized and reasons for misdiagnosis and incorrect mistreatment were analyzed. Results: Clinical symptoms were mainly polypnea and coughing after activities. And the chest high-resolution CT (HRCT) showed bilateral diffuse ground-glass opacities in 6 cases, a geographic pattern in 5 cases, a crazy paving pattern in 3 cases and air bronchogram in 2 cases. The misdiagnosis rate was high only with chest CT:3 cases were misdiagnosed as pneumonia, 1 case as pulmonary tuberculosis and 1 case as idiopathic pulmonary interstitial fibrosis, prior to admission. 6 patients were diagnosed by bronchoalveolar lavage fluid(BALF)and transbronchial lung biopsy(TBLB). In addition, their symptoms were markedly relived with BALF. Conclusion:Clinical manifestations of PAP were not specific, so its diagnosis was difficult and misdiagnosis and incorrect treatment were very likely if only based on HRCT. PAP should be considered when patients had polypnea and chest HRCT showed a geographic pattern and crazy paving pattern after activities. BALF was the most effective treatment. Blind treatment should be avoided when the diagnosis was not definite.%目的:研究肺泡蛋白沉积症的临床特征,诊治方法,提高临床诊治水平,减少误诊、误治.方法:分析近9年来收治患者的临床特征,肺部影像学表现,总结诊治经验,分析误诊、误治原因.结果:患者多表现为活动后气促,咳嗽.胸部高分辨CT主要征象:双肺弥漫性磨玻璃影6例,呈地图样分布5例,铺路石征3例,支气管充气征2例.仅凭胸部CT,误诊率高,入院前3例误诊肺炎,1例误诊肺结核,1例误诊特发性肺间质纤维化.6例患者入院后经肺泡灌洗液、支气管肺活检确诊.肺泡灌洗治疗后症状改善.结论:肺泡蛋白沉积症临床表现缺乏特异性,诊断有一定困难,仅凭胸部高分辨CT检查,误诊、误治普遍.当患者有活动后气促,胸部HRCT呈地图样、铺路石样改变时,需考虑PAP.肺泡灌洗是最有效治疗方法.诊断不清时避免盲目治疗.