期刊论文详细信息
BMC Pulmonary Medicine
Whole lung lavage combined with Granulocyte-macrophage colony stimulating factor inhalation for an adult case of refractory pulmonary alveolar proteinosis
Hui Huang1  Zuo jun Xu1  Yan xun Wang1  Xue feng Sun1  Hong yan Yu1 
[1] Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, #1 Shuaifuyuan Street, Dongcheng District, 100730 Beijing, China
关键词: Combination therapy;    Whole lung lavage (WLL);    Plasmapheresis;    GM-CSF inhalation;    Refractory pulmonary alveolar proteinosis (PAP);   
Others  :  862899
DOI  :  10.1186/1471-2466-14-87
 received in 2014-02-23, accepted in 2014-05-06,  发布年份 2014
PDF
【 摘 要 】

Background

Whole-lung lavage (WLL) is classically the first-line treatment for symptomatic pulmonary alveolar proteinosis (PAP). However, some patients require multiple WLLs because of refractory nature of their PAP. In this instance, these patients may benefit from new treatment regimens, and new therapies should be tried for these patients.

Case presentation

We describe a 47-year-old Chinese woman who was confidently diagnosed with pulmonary alveolar proteinosis (PAP) after bronchoalveolar lavage and transbronchial lung biopsy. The patient received four sessions of bilateral whole lung lavage (WLL) and one session of WLL in combination with plasmapheresis, each only producing short-term symptomatic relief. The patient was given a trial of combination therapy, which consisted of WLL and Granulocyte-macrophage colony-stimulating factor (GM-CSF) inhalation. The patient showed a gradual improvement in oxygenation and her daily activity, as well as a dramatic improvement in her pulmonary CT examination.

Conclusion

Bilateral WLL, in combination with GM-CSF inhalation, may be an effective treatment option for severe refractory PAP.

【 授权许可】

   
2014 Yu et al.; licensee BioMed Central Ltd.

【 预 览 】
附件列表
Files Size Format View
20140725022548172.pdf 3514KB PDF download
99KB Image download
【 图 表 】

【 参考文献 】
  • [1]Khan A, Agarwal R: Pulmonary alveolar proteinosis. Respir Care 2011, 56:1016-1028.
  • [2]Ramirez J, Kieffer RJ, Ball WJ: Bronchopulmonary lavage in man. Ann Intern Med 1965, 63:819-828.
  • [3]Campo I, Mariani F, Rodi G, Paracchini E, Tsana E, Piloni D, Nobili I, Kadija Z, Corsico A, Cerveri I, Chalk C, Trapnell BC, Braschi A, Tinelli C, Luisetti M: Assessment and management of pulmonary alveolar proteinosis in a reference center. Orphanet J Rare Dis 2013, 8:40. BioMed Central Full Text
  • [4]Beccaria M, Luisetti M, Rodi G, Corsico A, Zoia MC, Colato S, Pochetti P, Braschi A, Pozzi E, Cerveri I: Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis. Eur Respir J 2004, 23:526-531.
  • [5]Shah PL, Hansell D, Lawson PR, Reid KB, Morgan C: Pulmonary alveolar proteinosis: Clinical aspects and current concepts on pathogenesis. Thorax 2000, 55:67-77.
  • [6]Kitamura T, Tanaka N, Watanabe J, Uchida , Kanegasaki S, Yamada Y, Nakata K: Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor. J Exp Med 1999, 190:875-880.
  • [7]Sakagami T, Uchida K, Suzuki T, Carey BC, Wood RE, Wert SE, Whitsett JA, Trapnell BC, Luisetti M: Human GM-CSF autoantibodies and reproduction of pulmonary alveolar proteinosis. N Engl J Med 2009, 361:2679-2681.
  • [8]Sakagami T, Beck D, Uchida K, Suzuki T, Carey BC, Nakata K, Keller G, Wood RE, Wert SE, Ikegami M, Whitsett JA, Luisetti M, Davies S, Krischer JP, Brody A, Ryckman F, Trapnell BC: Patient-derived granulocyte/macrophage colony-stimulating factor autoantibodies reproduce pulmonary alveolar proteinosis in nonhuman primates. Am J Respir Crit Care Med 2010, 182:49-61.
  • [9]Bonfield TL, Kavuru MS, Thomassen MJ: Anti-GM-CSF titer predicts response to GM-CSF therapy in pulmonary alveolar proteinosis. Clin Immunol 2002, 105:342-350.
  • [10]Luisetti M, Rodi G, Perotti C, Campo I, Mariani F, Pozzi E, Trapnell BC: Plasmapheresis for treatment of pulmonary alveolar proteinosis. Eur Respir J 2009, 33:1220-1222.
  • [11]Khan A, Agarwal R, Aggarwal AN: Effectiveness of granulocyte-macrophage colony-stimulating factor therapy in autoimmune pulmonary alveolar proteinosis: A meta-analysis of observational studies. Chest 2012, 141:1273-2128.
  • [12]Venkateshiah SB, Yan TD, Bonfield TL, Thomassen MJ, Meziane M, Czich C, Kavuru MS: An open-label trial of granulocyte macrophage colony stimulating factor therapy for moderate symptomatic pulmonary alveolar proteinosis. Chest 2006, 130:227-323.
  文献评价指标  
  下载次数:17次 浏览次数:24次