【摘要】 目的提高肺泡蛋白沉积症(PAP)临床诊治水平。方法回顾性分析北京协和医院1990年1月至2007年10月确诊的PAP患者在院内外的误诊、误治过程,分析其原因。结果84例PAP患者平均诊断周期14.5个月。入院前67%患者存在误诊。近年来误诊为肺结核者减少,误诊为特发性间质性肺炎者增多。PAP患者在院外行支气管镜、肺泡灌洗(BAL)及经支气管肺活检(TBLB)的比例低,患者是否行BAL及TBLB检查与其是否误诊相关。87%的患者通过支气管镜、BAL及TBLB确诊。33.3%的患者在院外曾接受糖皮质激素治疗,经激素治疗的患者有6例合并严重感染,2例死亡。结论PAP患者误诊误治仍很普遍,误诊考虑可能与支气管镜、BAL、TBLB检查比例低有关。应重视PAP的影像学特征,积极行支气管镜、BAL、TBLB检查,避免误诊。诊断不明确时务必慎用激素。更多还原

【Abstract】 Objective To describe and analyze the misdiagnosis and mistherapy status of pulmonary alveolar proteinosis(PAP).Methods The misdiagnosis and mistherapy of PAP patients before admitting to PUMCH were analyzed retrospectively.Results In the 84 PAP patients,the average time from onset to diagnosis is 14.5 months.67% of these cases have experienced misdiagnosis before admission.Recently,the cases misdiagnosed with pulmonary tuberculosis tended to decrease and which with idiopathic interstitial pneumonia tended to increase.The percentage of the patients who had taken the bronchoscopic examination,bronchoalveolar lavage(BAL)and transbronchial lung biopsy(TBLB)was low.Taken the BAL/TBLB examination or not was significantly correlated with the misdiagnosis.The PAP diagnosis was established through bronchoscopic examination(including BAL and TBLB)in 87% patients.33.3% patients received corticosteroid therapy,in which 6 patients got a combination of severe infection at the time of admission,2 of them died.Conclusion The misdiagnosis and mistreatment happened commonly in PAP patients.It has some correlation with the decrease of the percentage of bronchoscopic examination and BAL and TBLB examination.The HRCT feature and the examination of bronchoscopy and BAL and TBLB is the key point in avoiding misdiagnosis.The administration of corticosteroids before diagnosis should be avoided.更多还原