切换至 "中华医学电子期刊资源库"

中华肺部疾病杂志(电子版) ›› 2026, Vol. 19 ›› Issue (04) : 714 -716. doi: 10.3877/cma.j.issn.1674-6902.2026.04.030

病例报告

全肺灌洗联合重组人GM-CSF雾化吸入治疗自身免疫性肺泡蛋白沉积症合并Ⅰ型呼吸衰竭一例
李茂思, 孙晓容(), 任茂玲, 刘佳铭   
  1. 400037 重庆,陆军(第三)军医大学第二附属医院呼吸与危重症医学科
  • 收稿日期:2026-03-25 出版日期:2026-08-25
  • 通信作者: 孙晓容

One case of autoimmune pulmonary alveolar proteinosis complicated with type i respiratory failure treated by whole-body lavage combined with recombinant human GM-CSF nebulization

Maosisi Li, Xiaorong Sun(), Maoling Ren   

  • Received:2026-03-25 Published:2026-08-25
  • Corresponding author: Xiaorong Sun
引用本文:

李茂思, 孙晓容, 任茂玲, 刘佳铭. 全肺灌洗联合重组人GM-CSF雾化吸入治疗自身免疫性肺泡蛋白沉积症合并Ⅰ型呼吸衰竭一例[J/OL]. 中华肺部疾病杂志(电子版), 2026, 19(04): 714-716.

Maosisi Li, Xiaorong Sun, Maoling Ren. One case of autoimmune pulmonary alveolar proteinosis complicated with type i respiratory failure treated by whole-body lavage combined with recombinant human GM-CSF nebulization[J/OL]. Chinese Journal of Lung Diseases(Electronic Edition), 2026, 19(04): 714-716.

图1 4月11日患者CT影像学图,CT结果示可见双肺纹理增多,双肺可见弥漫磨玻璃影或铺路石样密度增高模糊影,双肺弥漫性病变。图A为气管层面;图B为隆突层面;图C为肺动脉杈层面;图D为四腔心层面
图2 部分肺泡腔内充满块状无定形嗜伊红蛋白样物质,特殊染色标记PAS(局灶+),可符合肺泡蛋白沉积症改变注:(×20倍)
图3 4月29日患者CT影像学图的气管层面、隆突层面、肺动脉杈层面、四腔心层面,CT结果示双肺散在少量实性结节,较大者位于左肺下叶外基底段(Se203,Im257)见实性结节,大小约为7 mm×5 mm,双肺纹理增多,双肺可见弥漫磨玻璃影或铺路石样密度增高模糊影,与2025年4月11日结果相比较:显示双肺病变区密度较前稍降低。图A为气管层面;图B为隆突层面;图C为肺动脉杈层面;图D为四腔心层面
1
Trapnell BC, Nakata K, Bonella F, et al. Pulmonary alveolar proteinosis[J]. Nat Rev Dis Primers, 2019, 5(1): 16.
2
Jouneau S, Chauvin P, Lederlin M, et al. Pharmacotherapy for autoimmune pulmonary alveolar proteinosis[J]. Drugs, 2025, 85(10): 1193-1206.
3
Bonfield TL, Raychaudhuri B, Malur A, et al. PU.1 regulation of human alveolar macrophage differentiation requires granulocyte-macrophage colony-stimulating factor[J]. Am J Physiol Lung Cell Mol Physiol, 2003, 285(5): L1132-1136.
4
Inoue Y, Trapnell BC, Tazawa R, et al. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan[J]. Am J Respir Crit Care Med, 2008, 177(7): 752-762.
5
Holbert JM, Costello P, Li W, et al. CT features of pulmonary alveolar proteinosis[J]. AJR Am J Roentgenol, 2001, 176(5): 1287-1294.
6
Wells AU. The clinical utility of bronchoalveolar lavage in diffuse parenchymal lung disease[J]. Eur Respir Rev, 2010, 19(117): 237-241.
7
Mccarthy C, Bonella F, O′Callaghan M, et al. European respiratory society guidelines for the diagnosis and management of pulmonary alveolar proteinosis[J]. Eur Respir J, 2024, 64(5): 2400725.
8
Whitsett JA, Wert SE, Weaver TE. Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease[J]. Annu Rev Med, 2010, 61: 105-119.
9
Harari S, Caminati A. Update on diffuse parenchymal lung disease[J]. Eur Respir Rev, 2010, 19(116): 97-108.
10
Ferretti A, Fortwendel JR, Gebb SA, et al. Autoantibody-mediated pulmonary alveolar proteinosis in Rasgrp1-deficient mice[J]. J Immunol, 2016, 197(2): 470-479.
11
Uchida K, Nakata K, Carey B, et al. Standardized serum GM-CSF autoantibody testing for the routine clinical diagnosis of autoimmune pulmonary alveolar proteinosis[J]. J Immunol Methods, 2014, 402(1-2): 57-70.
12
Johkoh T, Itoh H, Müller NL, et al. Crazy-paving appearance at thin-section CT: Spectrum of disease and pathologic findings[J]. Radiology, 1999, 211(1): 155-160.
13
Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: Progress in the first 44 years[J]. Am J Respir Crit Care Med, 2002, 166(2): 215-235.
14
McCarthy C, Carey B, Trapnell BC. Blood testing for differential diagnosis of pulmonary alveolar proteinosis syndrome[J]. Chest, 2019, 155(2): 450-452.
15
Kitamura T, Uchida K, Tanaka N, et al. Serological diagnosis of idiopathic pulmonary alveolar proteinosis[J]. Am J Respir Crit Care Med, 2000, 162(2 Pt 1): 658-662.
16
Uchida K, Nakata K, Suzuki T, et al. Granulocyte/macrophage-colony-stimulating factor autoantibodies and myeloid cell immune functions in healthy subjects[J]. Blood, 2009, 113(11): 2547-2556.
17
Kusakabe Y, Uchida K, Hiruma T, et al. A standardized blood test for the routine clinical diagnosis of impaired GM-CSF signaling using flow cytometry[J]. J Immunol Methods, 2014, 413: 1-11.
18
Gay P, Wallaert B, Nowak S, et al. Efficacy of whole-lung lavage in pulmonary alveolar proteinosis: a multicenter international study of GELF[J]. Respiration, 2017, 93(3): 198-206.
19
Awab A, Khan MS, Youness HA. Whole lung lavage-technical details, challenges and management of complications[J]. J Thorac Dis, 2017, 9(6): 1697-1706.
20
Tazawa R, Ueda T, Abe M, et al. Inhaled GM-CSF for pulmonary alveolar proteinosis[J]. N Engl J Med, 2019, 381(10): 923-932.
[1] 张霞, 冯娅娆, 罗寰, 杨金良, 张斌, 郑学军. 尪痹胶囊联合来氟米特对类风湿关节炎炎症指标的影响[J/OL]. 中华关节外科杂志(电子版), 2025, 19(01): 55-64.
[2] 杨雅兰, 王晓华, 蔡宇航, 向东, 邝敏婷, 药晨, 肖漓, 巨春蓉. 肺移植治疗终末期肺朗格汉斯细胞组织细胞增生症一例[J/OL]. 中华移植杂志(电子版), 2025, 19(02): 114-116.
[3] 徐飞, 周楫, 赵欣, 王丹妮, 钱倩, 孙文逵. VV-ECMO下全肺灌洗术治疗肺泡蛋白沉积症一例并文献复习[J/OL]. 中华肺部疾病杂志(电子版), 2026, 19(04): 710-713.
[4] 冷法宁, 顾鹏. 肺泡蛋白沉积症一例[J/OL]. 中华肺部疾病杂志(电子版), 2020, 13(01): 109-111.
[5] 张建梅, 刘发盛, 陈欣, 朱振华. 新生儿高胆红素血症不同光照治疗方法与免疫变化的研究[J/OL]. 中华临床医师杂志(电子版), 2020, 14(02): 94-98.
阅读次数
全文


摘要


AI


AI小编
你好!我是《中华医学电子期刊资源库》AI小编,有什么可以帮您的吗?