肺泡蛋白沉积症患者及合并严重感染的病例临床分析

目的 通过分析肺泡蛋白沉积症(PAP)及合并严重感染的PAP患者误诊、误治情况及其原因,以提高PAP的临床诊治水平.方法 回顾性分析确诊的PAP患者84例及其中6例合并严重感染的PAP患者的临床表现,入院前的误诊、误治情况及后果.结果 84例PAP患者中66.7%(56/84)存在误诊,其中32.1%(27/84)误诊为特发性问质性肺炎(IIP),14.3%(12/84)误诊为肺结核.88.1%(74/84)患者曾接受抗感染治疗,33.3%(28/84)患者曾接受糖皮质激素治疗,19.0%(16/84)曾接受抗痨治疗.患者在院外行支气管镜检查率仅为53.6%(45/84).84例患者中86.9%(73/84)通过支气管镜、支气管肺泡灌洗(BAL)及经支气管镜肺活检(TBLB)确诊.确诊PAP时有6例患者合并严重感染,全部曾误诊为IIP并接受糖皮质激素治疗,2例死亡.结论 PAP患者误诊、误治仍很普遍,误用糖皮质激素治疗导致部分患者出现严重感染.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糖皮质激素.

Clinical analysis of pulmonary alveolar proteinosis and cases with combination of severe infection

Objective To describe and analyze the misdiagnosis and mistreatment status of pulmonary alveolar proteinosis (PAP), especially cases with combination of severe infection. Method The misdiagnosis and mistreatment of 84 PAP patients and 6 cases with combination of severe infection before admitting to PUMCH was analyzed retrospectively. Results 66.7%(56/84) had experienced misdiagnosis before admission. 32.1% (27/84) were misdiagnosed with idiopathic interstitial pneumonia (IIP) and 14.3% (12/84) with pulmonary tuberculosis. 88.1%(74/84) had received anti-infection treatment, 33.3%(28/84) received eortieosteroids, and 19.0%(16/84) received anti-tuberculosis treatment. The percentage of the pa-tients who had taken the bronchoscopic examination outside the PUMCH was 53.6%(45/84). The PAP pa-tients diagnosis was established through bronchoscopic examination (including BAL and TBLB) in 86.9% (73/84) patients. There were 6 patients who had severe infection when PAP established. All of these 6 cases had been misdiagnosed with IIP and received corticosteroids treatment, 2 of whom died. Conclusions The misdiagnosis and mistreatment happens commonly in PAP patients. The misuse of corticosteroids makes some PAP cases get the severe infection. The image feature and the examination of bronchoscopy, BAL and TBLB are the key point in avoiding misdiagnosis. The administration of corticosteroids in uncertain patients should be avoided.