肺泡蛋白沉积症15例临床分析及全肺灌洗疗效观察

目的探讨肺泡蛋白沉积症(PAP)的临床表现、影像学特征及评估全肺灌洗治疗效果。方法回顾性分析2009～2011年经病理证实的15例PAP患者临床表现、影像学特征及全肺灌洗术前后胸部CT、血气及肺功能变化。结果 PAP患者误诊率较高,其中5例考虑为职业相关继发性PAP,临床症状主要为咳嗽、胸闷、气喘,部分患者出现LDH、肿瘤指标升高,特征性影像学表现为地图样分布、铺路石征,行全肺灌洗术的6例患者临床症状及肺功能明显改善。结论特征性影像学表现有助于PAP的早期诊断,全肺灌洗术是PAP最有效治疗方法。

Clinical analysis of 15 patients with pulmonary alveolar proteinosis and effect of whole lung lavage treatment

Objective To analyze the clinical and radiological characteristics of pulmonary alveolar proteinosis(PAP),and evaluate the effect of whole lung lavage treatment.Methods Clinical data of 15 patients(2009~2011) with confirmed PAP were analyzed retrospectively.Results The rate of misdiagnosis of PAP was high.5 patients had a history of metal dusts and special chemical substance exposure,and were diagnosed as secondary PAP.Clinical symptoms were atypical,dyspnea after exertion and cough were the main complains.Levels of LDH and tumor markers in blood increased in some cases.Geographic and crazy paving were the typical radiological characteristics.6 patients were treated with whole lung lavage treatment,both the clinical characteristic and the pulmonary function were improved significantly.Conclusion The characteristic changes on chest CT is useful in early diagnosis of PAP,and the whole lung lavage is the most effective and standard treatment of PAP so far.