肺泡蛋白沉积症误诊分析及文献复习

Misdiagnosis and Literature Review of Pulmonary Alveolar Proteinosis

目的探讨肺泡蛋白沉积症(PAP)的临床特点、诊治措施及误诊原因、防范措施。方法对2020年1月—2023年6月收治的曾误诊的影像学呈不典型磨玻璃样表现的PAP 4例的临床资料进行回顾性分析。结果本组3例因咳嗽、咳痰伴或不伴发热就诊,1例体检发现双肺弥漫性病变就诊。胸部CT检查皆提示弥漫性磨玻璃样表现。就诊初期均误诊为肺炎,给予相应治疗效果不佳,误诊时间10 d~4年。4例最终均由支气管肺泡灌洗液过碘酸雪夫(PAS)染色后确诊为PAP。2例接受大剂量全肺肺泡灌洗治疗,2例给予对症支持治疗,均病情好转出院。结论PAP为罕见病,而以单纯大片状磨玻璃影为主要影像学表现者更为少见,临床上需依靠病史、临床表现、影像表现、支气管镜、支气管肺泡灌洗液PAS染色综合分析进行诊断,积极治疗多预后良好。

Objective To investigate the clinical features,diagnosis and treatment measures,causes of misdiagnosis and preventive measures of pulmonary alveolar proteinosis(PAP).Methods From January 2020 to June 2023,the clinical data of 4 patients with PAP who had been misdiagnosed and showed atypical ground-glass appearance were retrospectively analyzed.Results In this group,3 cases presented with cough,sputum with or without fever,and 1 case was found to have diffuse lesions of both lungs.CT examination of the chest indicated diffuse ground glass appearance.The initial diagnosis was misdiagnosed as pneumonia,and the corresponding treatment was not effective,and the duration of misdiagnosis was 10 d-4 years.All 4 cases were eventually diagnosed as PAP after bronchoalveolar lavage fluid periodic acid-Schiff(PAS)staining.Two cases received large dose of whole lung alveolar lavage,and two cases received symptomatic supportive treatment.They were discharged after improvement.Conclusion PAP is a rare disease,and it is even rarer to have a single large flaker-like ground glass image as the main imaging manifestation.Clinical diagnosis should be made by comprehensive analysis of history,clinical manifestations,imaging manifestations,bronchoscopy,and PAS staining in alveolar lavage.Active treatment usually leads to a good prognosis.