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A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy

Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the accumulation of alveolar surfactants due to dysfunction of granulocyte-macrophage colony-stimulating factor-dependent cholesterol clearance. Whole-lung lavage is the current standard of care for PAP, but it can lead...

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Autores principales: Takano, Tomotsugu, Takeda, Keisuke, Nakamura, Satoshi, Akiyama, Genta, Ando, Nobuhisa, Komori, Masashi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9190053/
https://www.ncbi.nlm.nih.gov/pubmed/35707405
http://dx.doi.org/10.1016/j.rmcr.2022.101684
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author Takano, Tomotsugu
Takeda, Keisuke
Nakamura, Satoshi
Akiyama, Genta
Ando, Nobuhisa
Komori, Masashi
author_facet Takano, Tomotsugu
Takeda, Keisuke
Nakamura, Satoshi
Akiyama, Genta
Ando, Nobuhisa
Komori, Masashi
author_sort Takano, Tomotsugu
collection PubMed
description Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the accumulation of alveolar surfactants due to dysfunction of granulocyte-macrophage colony-stimulating factor-dependent cholesterol clearance. Whole-lung lavage is the current standard of care for PAP, but it can lead to the exacerbation of hypoxia. A medication targeting cholesterol homeostasis is a promising therapy for refractory PAP. We present a case of autoimmune PAP with severe hypoxia that was successfully treated with segmental lung lavage (SLL). Following SLL for disease relapse, statin treatment for dyslipidemia was started. After initiating statin treatment, the patient did not require bronchoalveolar lavage for 10 months.
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spelling pubmed-91900532022-06-14 A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy Takano, Tomotsugu Takeda, Keisuke Nakamura, Satoshi Akiyama, Genta Ando, Nobuhisa Komori, Masashi Respir Med Case Rep Case Report Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the accumulation of alveolar surfactants due to dysfunction of granulocyte-macrophage colony-stimulating factor-dependent cholesterol clearance. Whole-lung lavage is the current standard of care for PAP, but it can lead to the exacerbation of hypoxia. A medication targeting cholesterol homeostasis is a promising therapy for refractory PAP. We present a case of autoimmune PAP with severe hypoxia that was successfully treated with segmental lung lavage (SLL). Following SLL for disease relapse, statin treatment for dyslipidemia was started. After initiating statin treatment, the patient did not require bronchoalveolar lavage for 10 months. Elsevier 2022-06-06 /pmc/articles/PMC9190053/ /pubmed/35707405 http://dx.doi.org/10.1016/j.rmcr.2022.101684 Text en © 2022 The Authors https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Takano, Tomotsugu
Takeda, Keisuke
Nakamura, Satoshi
Akiyama, Genta
Ando, Nobuhisa
Komori, Masashi
A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy
title A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy
title_full A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy
title_fullStr A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy
title_full_unstemmed A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy
title_short A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy
title_sort case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9190053/
https://www.ncbi.nlm.nih.gov/pubmed/35707405
http://dx.doi.org/10.1016/j.rmcr.2022.101684
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