摘要
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。
[Abstract] Objective Pulmonary alveolar proteinosis is a rare disease, Misdiagnosis frequently happen because of unfamiliarity with the disease. However, prognosis is determined by correct diagnosis, whole or segmental lung lavage therapy will improve the prognosis. We summarized our experience and reviewed the recent literature. Methods The data of 15 cases of pulmonary alveolar proteinosis from 1990 to April 2000 in Peking Union Medical College Hospital were analysed retrospectively .Results There were 15 cases of pulmonary alveolar proteinosis (male: female 9 ∶ 6) with an average age of 39.2±2.1 years old. Eight cases among them had history of dust exposure. Dyspnea is the most common symptom, cough and expectoration are also common. 38.5% (5 / 13) have increased lactate dehydrogenase level. The level of lactate dehydrogenase is correlated with alveolar/arterial pressure difference (r =0.81, P =0.0013) . Chest film or CT suggests diffuse consolidation. Pulmonary function changes include restrictive pattern, decreased diffusion capacity and hypoxemia. Diagnostic bronchoalveolar lavage and trans-bronchial lung biopsy are most frequently used to establish the diagnosis. Whole or segmental lung lavage is the only treatment. Pulmonary functions are improved significantly after lung lavage treatment. The understanding of the role of granulocyte - macrophage colony - stimulating factor (GM- CSF) in alveolar proteinosis in recent years will possibly provide an alternative treatment with GM - CSF. Conclusion Pulmonary alveolar proteinosis is rare, but the diagnosis is not difficult if we are familiar with the syndrome. Whole lung lavage is the treatment of choice.
