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Published in: BMC Pulmonary Medicine 1/2014

Open Access 01-12-2014 | Case report

Whole lung lavage combined with Granulocyte-macrophage colony stimulating factor inhalation for an adult case of refractory pulmonary alveolar proteinosis

Authors: Hong yan Yu, Xue feng Sun, Yan xun Wang, Zuo jun Xu, Hui Huang

Published in: BMC Pulmonary Medicine | Issue 1/2014

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Abstract

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.
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Literature
1.
go back to reference Khan A, Agarwal R: Pulmonary alveolar proteinosis. Respir Care. 2011, 56: 1016-1028. 10.4187/respcare.01125.CrossRefPubMed Khan A, Agarwal R: Pulmonary alveolar proteinosis. Respir Care. 2011, 56: 1016-1028. 10.4187/respcare.01125.CrossRefPubMed
2.
go back to reference Ramirez J, Kieffer RJ, Ball WJ: Bronchopulmonary lavage in man. Ann Intern Med. 1965, 63: 819-828. 10.7326/0003-4819-63-5-819.CrossRefPubMed Ramirez J, Kieffer RJ, Ball WJ: Bronchopulmonary lavage in man. Ann Intern Med. 1965, 63: 819-828. 10.7326/0003-4819-63-5-819.CrossRefPubMed
3.
go back to reference 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: 40-10.1186/1750-1172-8-40.CrossRefPubMedPubMedCentral 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: 40-10.1186/1750-1172-8-40.CrossRefPubMedPubMedCentral
4.
go back to reference 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. 10.1183/09031936.04.00102704.CrossRefPubMed 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. 10.1183/09031936.04.00102704.CrossRefPubMed
5.
go back to reference 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. 10.1136/thorax.55.1.67.CrossRefPubMedPubMedCentral 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. 10.1136/thorax.55.1.67.CrossRefPubMedPubMedCentral
6.
go back to reference 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. 10.1084/jem.190.6.875.CrossRefPubMedPubMedCentral 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. 10.1084/jem.190.6.875.CrossRefPubMedPubMedCentral
7.
go back to reference 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. 10.1056/NEJMc0904077.CrossRefPubMedPubMedCentral 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. 10.1056/NEJMc0904077.CrossRefPubMedPubMedCentral
8.
go back to reference 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. 10.1164/rccm.201001-0008OC.CrossRefPubMedPubMedCentral 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. 10.1164/rccm.201001-0008OC.CrossRefPubMedPubMedCentral
9.
go back to reference 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. 10.1006/clim.2002.5301.CrossRefPubMed 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. 10.1006/clim.2002.5301.CrossRefPubMed
10.
go back to reference 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. 10.1183/09031936.00097508.CrossRefPubMed 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. 10.1183/09031936.00097508.CrossRefPubMed
11.
go back to reference 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. 10.1378/chest.11-0951.CrossRefPubMed 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. 10.1378/chest.11-0951.CrossRefPubMed
12.
go back to reference 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. 10.1378/chest.130.1.227.CrossRefPubMed 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. 10.1378/chest.130.1.227.CrossRefPubMed
Metadata
Title
Whole lung lavage combined with Granulocyte-macrophage colony stimulating factor inhalation for an adult case of refractory pulmonary alveolar proteinosis
Authors
Hong yan Yu
Xue feng Sun
Yan xun Wang
Zuo jun Xu
Hui Huang
Publication date
01-12-2014
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2014
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/1471-2466-14-87

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