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Published in: Journal of Medical Case Reports 1/2015

Open Access 01-12-2015 | Case report

Marked improvement in autoimmune pulmonary alveolar proteinosis with severe hypoxemia in a patient treated with ambroxol: a case report

Authors: Nao Oda, Koji Tamai, Yujiro Suzuki, Harukazu Yoshimatsu, Hirofumi Matsuoka, Yusuke Matsumoto, Nobuhiko Okada

Published in: Journal of Medical Case Reports | Issue 1/2015

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Abstract

Introduction

Pulmonary alveolar proteinosis is characterized by accumulation of surfactant and phospholipids in the pulmonary alveoli. Whole lung lavage is considered the first-line therapy, which requires special techniques. To the best of our knowledge, there have only been limited reports that have demonstrated the effectiveness of ambroxol on a mild case of pulmonary alveolar proteinosis.

Case presentation

A 72-year-old Japanese woman presented to our hospital with a one-year history of productive cough and progressive dyspnea. Her chest computed tomography scan showed a bilateral crazy-paving pattern in both of her lungs. She was diagnosed with autoimmune pulmonary alveolar proteinosis based on bronchoalveolar lavage findings and the presence of serum anti-granulocyte macrophage colony-stimulating factor antibodies. She was severely hypoxemic, so we recommended whole lung lavage or inhaled granulocyte macrophage colony-stimulating factor treatment, which she refused. We initiated treatment with ambroxol and her symptoms markedly improved.

Conclusions

Although whole lung lavage is the first-line therapy for pulmonary alveolar proteinosis, oral ambroxol could be an alternative treatment option, even in patients with severe respiratory compromise.
Literature
1.
go back to reference Leth S, Bendstrup E, Vestergaard H, Hilberg O. Autoimmune pulmonary alveolar proteinosis: treatment options in year 2013. Respirology. 2013;18:82–91.CrossRefPubMed Leth S, Bendstrup E, Vestergaard H, Hilberg O. Autoimmune pulmonary alveolar proteinosis: treatment options in year 2013. Respirology. 2013;18:82–91.CrossRefPubMed
2.
go back to reference Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N, et al. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Am J Respir Crit Care Med. 2008;177:752–62.CrossRefPubMedPubMedCentral Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N, et al. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Am J Respir Crit Care Med. 2008;177:752–62.CrossRefPubMedPubMedCentral
3.
go back to reference Kitamura T, Tanaka N, Watanabe J, Uchida, Kanegasaki S, Yamada Y, et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor. J Exp Med. 1999;190:875–80.CrossRefPubMedPubMedCentral Kitamura T, Tanaka N, Watanabe J, Uchida, Kanegasaki S, Yamada Y, et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor. J Exp Med. 1999;190:875–80.CrossRefPubMedPubMedCentral
4.
go back to reference Kavuru MS, Malur A, Marshall I, Barna BP, Meziane M, Huizar I, et al. An open-label trial of rituximab therapy in pulmonary alveolar proteinosis. Eur Respir J. 2011;38:1361–7.CrossRefPubMed Kavuru MS, Malur A, Marshall I, Barna BP, Meziane M, Huizar I, et al. An open-label trial of rituximab therapy in pulmonary alveolar proteinosis. Eur Respir J. 2011;38:1361–7.CrossRefPubMed
5.
go back to reference Luisetti M, Rodi G, Perotti C, Campo I, Mariani F, Pozzi E, et al. Plasmapheresis for treatment of pulmonary alveolar proteinosis. Eur Respir J. 2009;33:1220–2.CrossRefPubMed Luisetti M, Rodi G, Perotti C, Campo I, Mariani F, Pozzi E, et al. Plasmapheresis for treatment of pulmonary alveolar proteinosis. Eur Respir J. 2009;33:1220–2.CrossRefPubMed
6.
go back to reference Hashizume T. Pulmonary alveolar proteinosis successfully treated with ambroxol. Int Med. 2002;41:1175–8.CrossRef Hashizume T. Pulmonary alveolar proteinosis successfully treated with ambroxol. Int Med. 2002;41:1175–8.CrossRef
7.
go back to reference Diaz JP, Manresa Presas F, Benasco C, Guardiola J, Munoz L, Clariana A, et al. Response to surfactant activator (ambroxol) in alveolar proteinosis. Lancet. 1984;1:1023.CrossRefPubMed Diaz JP, Manresa Presas F, Benasco C, Guardiola J, Munoz L, Clariana A, et al. Response to surfactant activator (ambroxol) in alveolar proteinosis. Lancet. 1984;1:1023.CrossRefPubMed
8.
go back to reference Dranoff G, Crawford AD, Sadelain M, Ream B, Rashid A, Bronson RT, et al. Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis. Science. 1994;264:713–6.CrossRefPubMed Dranoff G, Crawford AD, Sadelain M, Ream B, Rashid A, Bronson RT, et al. Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis. Science. 1994;264:713–6.CrossRefPubMed
9.
go back to reference Tazawa R, Inoue Y, Arai T, Takada T, Kasahara Y, Hojo M, et al. Duration of benefit in patients with autoimmune pulmonary alveolar proteinosis after inhaled granulocyte-macrophage colony-stimulating factor therapy. Chest. 2014;145:729–37.CrossRefPubMed Tazawa R, Inoue Y, Arai T, Takada T, Kasahara Y, Hojo M, et al. Duration of benefit in patients with autoimmune pulmonary alveolar proteinosis after inhaled granulocyte-macrophage colony-stimulating factor therapy. Chest. 2014;145:729–37.CrossRefPubMed
10.
go back to reference Mahut B, Delacourt C, Scheinmann P, de Blic J, Mani TM, Fournet JC, et al. Pulmonary alveolar proteinosis: experience with eight pediatric cases and a review. Pediatrics. 1996;97:117–22.PubMed Mahut B, Delacourt C, Scheinmann P, de Blic J, Mani TM, Fournet JC, et al. Pulmonary alveolar proteinosis: experience with eight pediatric cases and a review. Pediatrics. 1996;97:117–22.PubMed
11.
go back to reference Garcia Rio F, Alvarez-Sala R, Caballero P, Prados C, Pino JM, Villamor J. Six cases of pulmonary alveolar proteinosis: presentation of unusual associations. Monaldi Arch Chest Dis. 1995;50:12–5.PubMed Garcia Rio F, Alvarez-Sala R, Caballero P, Prados C, Pino JM, Villamor J. Six cases of pulmonary alveolar proteinosis: presentation of unusual associations. Monaldi Arch Chest Dis. 1995;50:12–5.PubMed
12.
go back to reference Luisetti M, Salmona M, Pozzi E, Genghini M, Spialtini L, Masturzo P. In vivo studies of rat alveolar macrophase microviscosity: influence of pulmonary surfactant synthesis stimulation. Lung. 1987;165:333–40.CrossRefPubMed Luisetti M, Salmona M, Pozzi E, Genghini M, Spialtini L, Masturzo P. In vivo studies of rat alveolar macrophase microviscosity: influence of pulmonary surfactant synthesis stimulation. Lung. 1987;165:333–40.CrossRefPubMed
13.
go back to reference Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med. 2002;166:215–35.CrossRefPubMed Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med. 2002;166:215–35.CrossRefPubMed
Metadata
Title
Marked improvement in autoimmune pulmonary alveolar proteinosis with severe hypoxemia in a patient treated with ambroxol: a case report
Authors
Nao Oda
Koji Tamai
Yujiro Suzuki
Harukazu Yoshimatsu
Hirofumi Matsuoka
Yusuke Matsumoto
Nobuhiko Okada
Publication date
01-12-2015
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2015
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-015-0588-6

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