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目的 肺泡蛋白沉积症(PAP)是一种少见病,临床由于认识不足,容易误诊,而PAP通过灌洗治疗常可获得良好预后。本文通过分析PAP病例,总结经验,提高认识,并结合文献讨论最新进展。方法 回顾性分析北京协和医院1990年至2000年4月间收治的15例PAP病人。结果 15例病人中男女之比为9:6,平均年龄39.2±2.1岁。其中8例有各种接触史。呼吸困难为最常见的症状,其次是咳嗽和咯痰。38.5％(5/13)有乳酸脱氢酶的升高,乳酸脱氢酶水平和P(A-a)O:值呈正相关(r=0.81,P=0.0013)。胸片和胸部CT检查主要表现为双肺弥漫性实变影。肺功能改变主要表现为限制性通气功能障碍、弥散障碍和低氧血症。经纤维支气管镜肺泡灌洗和肺活检是获得诊断的常用方法。治疗方法为分段或全肺灌洗,近年来,全肺灌洗治疗成为首选治疗方法。灌洗治疗后受损肺功能显著改善。近年来对粒单集落刺激因子(GM-CSF)在肺泡蛋白沉积症中发病机制的认识,使GM-CSF可能成为一种新的治疗选择。结论 肺泡蛋白沉积症虽然少见,但只要提高认识,诊断并不困难。治疗以全肺灌洗治疗为首选。
Objective Pulmonary alveolar proteinosis (PAP) is a rare disease that is often misdiagnosed due to lack of knowledge in the clinic. However, PAP often achieves a good prognosis after lavage treatment. This paper analyzes the PAP cases, summarize the experience and raise awareness, combined with the literature to discuss the latest developments. Methods A retrospective analysis of Peking Union Medical College Hospital from 1990 to April 2000 were treated 15 cases of PAP patients. Results The ratio of male to female in 15 patients was 9: 6, with an average age of 39.2 ± 2.1 years. There are 8 cases of various exposure history. Dyspnea was the most common symptom, followed by cough and expectoration. There was a positive correlation between lactate dehydrogenase level and P (A-a) O ratio in 38.5% (5/13) patients (r = 0.81, P = 0.0013). Chest radiography and chest CT showed mainly diffuse diffuse shadow of the lungs. Pulmonary function changes mainly manifested as restrictive ventilatory dysfunction, diffuse disorders and hypoxemia. Bronchoscopy bronchoalveolar lavage and lung biopsy are commonly used methods of diagnosis. Treatment for segmental or whole lung lavage, in recent years, the whole lung lavage treatment has become the preferred treatment. Impaired lung function after lavage treatment significantly improved. In recent years, understanding of the pathogenesis of GM-CSF in pulmonary alveolar proteinosis makes GM-CSF a new therapeutic option. Conclusions Although pulmonary alveolar proteinosis is rare, diagnosis is not difficult if it is to raise awareness. Treatment of whole lung lavage treatment is preferred.