肺移植治疗肺泡蛋白沉积症1例并文献复习

Lung transplantation for pulmonary alveolar proteinosis:a case report and literature review

目的探讨肺移植治疗终末期肺泡蛋白沉积症的疗效。方法患者女,55岁,因"胸闷、气喘伴咳嗽6年,加重1年"入院,经反复多次全肺灌洗治疗,效果欠佳,遂行同种异体序贯式双肺移植治疗,观察其临床资料并进行相关文献复习。以"肺移植"和"肺泡蛋白沉积症"为检索词检索中国知网及万方医学网中文文献数据库,以"lung transplantation"及"pulmonary alveolar proteinosis"为检索词检索PubMed数据库,检索时间截止到2022年2月。结果该患者既往行全肺灌洗19次,前期效果尚可,后期症状进行性加重,粒细胞-巨噬细胞集落刺激因子雾化吸入治疗后,出现过敏反应,胸部CT提示肺部病变逐渐加重。2022年2月15日行肺移植术,PaO_(2)从47 mmHg(1 mmHg=0.133 kPa)上升到85 mmHg,可脱离吸氧活动,2022年3月2日出院,随访病情稳定。文献检索获得相关英文文献13篇,其中论著2篇,病例报告11篇;肺移植后继发肺泡蛋白沉积症9篇;肺移植治疗肺泡蛋白沉积症4篇,报道了3个病例;国内未见肺移植治疗肺泡蛋白沉积症的报道。结论肺移植治疗终末期肺泡蛋白沉积症,早期安全有效,长期效果有待进一步观察。

Objective To observe the efficacy of lung transplantation for pulmonary alveolar proteinosis(PAP)patients and to improve the understanding of the therapy.Methods The clinical data of a patient with autoimmune PAP treated with sequential homogenous bilateral lung transplantation were described and the literatures were reviewed.Results This 55-year-old female patient was diagnosed with autoimmune PAP and had been treated with whole lung lavage for 19 times,but only achieved short-term symptomatic relief after each operation.Inhalation of granulocyte macrophage colony stimulating factor occurred allergic reactions.Lung transplantation was performed on February 15,2022,and a significant improvement in oxygenation and clinical symptoms were observed.The patient remained stable during follow-up.Conclusion Treatment with lung transplantation is safe and effective for end-stage patients with PAP in the early phase,but the long-term effect remains to be observed.