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Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by abnormal accumulation of surfactant in the alveoli. Whole lung lavage (WLL) is the standard treatment for severe autoimmune PAP (aPAP); however, it is highly invasive. Intrapulmonary percussive ventilation (IPV) is a non‐invasiv...

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Autores principales: Tashiro, Takahiro, Tomita, Yusuke, Inaba, Megumi, Hayashi, Kumiko, Hirata, Naomi, Sakagami, Takuro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7641663/
https://www.ncbi.nlm.nih.gov/pubmed/33173585
http://dx.doi.org/10.1002/rcr2.676
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author Tashiro, Takahiro
Tomita, Yusuke
Inaba, Megumi
Hayashi, Kumiko
Hirata, Naomi
Sakagami, Takuro
author_facet Tashiro, Takahiro
Tomita, Yusuke
Inaba, Megumi
Hayashi, Kumiko
Hirata, Naomi
Sakagami, Takuro
author_sort Tashiro, Takahiro
collection PubMed
description Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by abnormal accumulation of surfactant in the alveoli. Whole lung lavage (WLL) is the standard treatment for severe autoimmune PAP (aPAP); however, it is highly invasive. Intrapulmonary percussive ventilation (IPV) is a non‐invasive technique that delivers small bursts of high‐flow respiratory gas into the lung and mobilizes secretions. As IPV is beneficial for chronic respiratory diseases such as cystic fibrosis and bronchiectasis to reduce sputum, it was hypothesized that IPV will ameliorate aPAP by mobilizing and removing accumulated surfactant and foamy macrophages. Here, we report the case of a 52‐year‐old female with severe aPAP and progressive respiratory failure. She received intermittent IPV therapy for six months and thereby showed improvement in assessments of chest computed tomography (CT), lung function, and oxygenation. We suggest that IPV should be used as an alternative therapy for patients with aPAP and respiratory failure.
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spelling pubmed-76416632020-11-09 Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation Tashiro, Takahiro Tomita, Yusuke Inaba, Megumi Hayashi, Kumiko Hirata, Naomi Sakagami, Takuro Respirol Case Rep Case Reports Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by abnormal accumulation of surfactant in the alveoli. Whole lung lavage (WLL) is the standard treatment for severe autoimmune PAP (aPAP); however, it is highly invasive. Intrapulmonary percussive ventilation (IPV) is a non‐invasive technique that delivers small bursts of high‐flow respiratory gas into the lung and mobilizes secretions. As IPV is beneficial for chronic respiratory diseases such as cystic fibrosis and bronchiectasis to reduce sputum, it was hypothesized that IPV will ameliorate aPAP by mobilizing and removing accumulated surfactant and foamy macrophages. Here, we report the case of a 52‐year‐old female with severe aPAP and progressive respiratory failure. She received intermittent IPV therapy for six months and thereby showed improvement in assessments of chest computed tomography (CT), lung function, and oxygenation. We suggest that IPV should be used as an alternative therapy for patients with aPAP and respiratory failure. John Wiley & Sons, Ltd 2020-11-04 /pmc/articles/PMC7641663/ /pubmed/33173585 http://dx.doi.org/10.1002/rcr2.676 Text en © 2020 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology. This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Reports
Tashiro, Takahiro
Tomita, Yusuke
Inaba, Megumi
Hayashi, Kumiko
Hirata, Naomi
Sakagami, Takuro
Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation
title Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation
title_full Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation
title_fullStr Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation
title_full_unstemmed Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation
title_short Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation
title_sort severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7641663/
https://www.ncbi.nlm.nih.gov/pubmed/33173585
http://dx.doi.org/10.1002/rcr2.676
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