肺泡蛋白沉着症1例并文献复习

A case of alveolar protein deposition and literature review

目的探讨肺泡蛋白沉着症(PAP)发病原因、临床特征、肿瘤标志物的动态变化、大容量全肺灌洗术的临床疗效及安全性、提高临床医生对肺泡蛋白沉着症的认识、减少漏诊、误诊。方法回顾性分析1例肺泡蛋白沉着症病人2020年9月至2021年3月共3次在皖北煤电集团总医院呼吸与危重医学科住院的临床资料(包括吸烟史、职业暴露、临床表现、胸部影像学、肺功能、电子支气管镜检查、实验室检查、大容量全肺灌洗的临床疗效及安全性)。结果吸烟及职业暴露是PAP的易患因素之一,临床症状缺乏特异性;胸部高分辨率CT(HRCT)提示“铺路石征”,并呈“地图样”分布;肺功能提示一氧化碳(CO)弥散功能重度减低,限制性通气功能障碍;血气分析提示低氧血症并过度通气;肿瘤标志物癌胚抗原(CEA)轻度升高,细胞角蛋白19片段(CYFRA211)明显升高,随着病情好转,数值逐渐下降。共进行3次大容量双肺同期灌洗术,病人临床症状及影像学均明显好转。结论PAP是一种罕见的呼吸系统疾病,临床表现缺乏特异性,容易误诊,大容量全肺灌洗术治疗肺泡蛋白沉着症安全、有效、值得临床推广应用。

Objective To investigate the pathogenesis,clinical characteristics,dynamic changes in tumor markers,clinical efficacy and safety of large-volume whole-lung lavage,improve clinicians'understanding of alveolar proteinosis,and reduce missed diagnosis and misdiagnosis of pulmonary alveolar proteinosis(PAP).Methods Retrospective analysis of clinical data(including smoking history,occupational exposure,clinical manifestations,chest imaging,pulmonary function,2 electronic bronchoscopy,laboratory tests,clinical efficacy and safety of high volume whole lung lavage)of a patient with alveolar protein deposition who was admitted to the Department of Respiratory and Critical Care Medicine of the Wanbei Coal and Electric Power Group General Hospital on a total of three occasions from September 2020 to March 2021.Results Smoking and occupational exposure are factors that predispose individuals to PAP,and clinical symptoms are not specific.High-resolution CT of the chest(HRCT)indicated"paving stone signs"with a"map-like"distribution.Lung function suggested a severe reduction in carbon monoxide(CO)diffusion and restrictive ventilation.Blood gas analysis suggested hypoxemia and hyperventilation.Tumor marker carcinoembryonic antigen(CEA)was slightly increased,and cytokeratin 19 fragment(CYFRA211)was significantly increased,with values gradually decreasing as the condition improved.A total of 3 simultaneous high-volume double lung lavage procedures were performed,and the patient showed significant improvement in clinical symptoms and imaging.Conclusion PAP is a rare respiratory disease with a lack of specificity in clinical manifestations that is easily misdiagnosed.High-volume whole lung lavage is safe,effective and worthy of clinical application in the treatment of alveolar proteinosis.