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目的通过分析肺泡蛋白沉积症(PAP)病例,提高该病的诊疗水平,为PAP的临床研究提供参考。方法回顾性分析湖南省职业病防治院2008年5月至2015年6月确诊的34例PAP患者的临床资料。结果 34例中有18例(52.9%)患者有不同粉尘吸入史。咳嗽、进行性呼吸困难是PAP常见的临床症状。低氧血症和肺功能损害分别达到87.9%和90.9%。胸部高分辨CT呈铺路石样、地图样分布的特征性改变可提示诊断。34例患者均予以大容量全肺灌洗治疗,动脉血氧分压由(62.5±12.9)mm Hg上升至(76.0±12.2)mm Hg,DLCO占预计值百分比从(50.1±14.9)%上升至(64.6±14.4)%。动脉血氧和肺功能与灌洗前比较均明显改善(P<0.01)。有13例患者复发,复发率为38.2%。结论 PAP病因未明,粉尘吸入暴露与PAP的发病可能有关系。大容量全肺灌洗临床治疗效果显著,但容易复发。PAP病因治疗上还需进一步研究。
Objective To improve the diagnosis and treatment of pulmonary disease by analyzing the cases of pulmonary alveolar proteinosis (PAP) and provide a reference for the clinical research of PAP. Methods The clinical data of 34 patients with PAP diagnosed in Hunan Provincial Occupational Disease Prevention and Treatment Hospital from May 2008 to June 2015 were retrospectively analyzed. Results Of the 34 cases, 18 (52.9%) had different dust inhalation history. Cough, progressive dyspnea are common clinical symptoms of PAP. Hypoxemia and pulmonary dysfunction reached 87.9% and 90.9% respectively. Chest high-resolution CT was paving stone-like, map-like distribution of the characteristic changes can prompt diagnosis. All of the 34 patients were treated with high-volume total lavage. The partial pressure of arterial oxygen increased from (62.5 ± 12.9) mm Hg to (76.0 ± 12.2) mm Hg, and the percentage of expected DLCO increased from (50.1 ± 14.9)% to (64.6 ± 14.4)%. Arterial oxygenation and pulmonary function were significantly improved compared with those before lavage (P <0.01). Thirteen patients relapsed with a recurrence rate of 38.2%. Conclusions The etiology of PAP is unknown, and the inhalation of dust may have something to do with the pathogenesis of PAP. Large-capacity whole lung lavage clinical treatment of significant effect, but easy to relapse. PAP etiology treatment needs further study.