Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis

Hira Iftikhar,1 Girish B Nair,1 Anupam Kumar2 1Division of Pulmonary and Critical Care, Beaumont Health, OUWB School of Medicine, Royal Oak, MI, USA; 2Division of Pulmonary and Critical Care, Baylor College of Medicine, Houston, TX, USACorrespondence: Hira Iftikhar Tel +1 2485251389Email hiraiftikha...

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Autores principales:	Iftikhar H, Nair GB, Kumar A
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2021
Materias:	alveolar macrophage surfactant protein granulocyte macrophage-colony stimulating factor whole lung lavage lung transplant gene therapy rituximab gm-csf supplementation glucocorticoids Therapeutics. Pharmacology RM1-950
Acceso en línea:	https://doaj.org/article/6b77820b22084e70bc52870c56b4bc87
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spelling	oai:doaj.org-article:6b77820b22084e70bc52870c56b4bc872021-12-02T16:26:43ZUpdate on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis1178-203Xhttps://doaj.org/article/6b77820b22084e70bc52870c56b4bc872021-08-01T00:00:00Zhttps://www.dovepress.com/update-on-diagnosis-and-treatment-of-adult-pulmonary-alveolar-proteino-peer-reviewed-fulltext-article-TCRMhttps://doaj.org/toc/1178-203XHira Iftikhar,1 Girish B Nair,1 Anupam Kumar2 1Division of Pulmonary and Critical Care, Beaumont Health, OUWB School of Medicine, Royal Oak, MI, USA; 2Division of Pulmonary and Critical Care, Baylor College of Medicine, Houston, TX, USACorrespondence: Hira Iftikhar Tel +1 2485251389Email hiraiftikhar@live.comAbstract: Pulmonary alveolar proteinosis (PAP) is a rare pulmonary surfactant homeostasis disorder resulting in buildup of lipo-proteinaceous material within the alveoli. PAP is classified as primary (autoimmune and hereditary), secondary, congenital and unclassifiable type based on the underlying pathogenesis. PAP has an insidious onset and can, in some cases, progress to severe respiratory failure. Diagnosis is often secured with bronchoalveolar lavage in the setting of classic imaging findings. Recent insights into genetic alterations and autoimmune mechanisms have provided newer diagnostics and treatment options. In this review, we discuss the etiopathogenesis, diagnosis and treatment options available and emerging for PAP.Keywords: alveolar macrophage, surfactant protein, granulocyte macrophage-colony stimulating factor, whole lung lavage, lung transplant, gene therapy, alveolar proteinosisIftikhar HNair GBKumar ADove Medical Pressarticlealveolar macrophagesurfactant proteingranulocyte macrophage-colony stimulating factorwhole lung lavagelung transplantgene therapyrituximabgm-csf supplementationglucocorticoidsTherapeutics. PharmacologyRM1-950ENTherapeutics and Clinical Risk Management, Vol Volume 17, Pp 701-710 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	alveolar macrophage surfactant protein granulocyte macrophage-colony stimulating factor whole lung lavage lung transplant gene therapy rituximab gm-csf supplementation glucocorticoids Therapeutics. Pharmacology RM1-950
spellingShingle	alveolar macrophage surfactant protein granulocyte macrophage-colony stimulating factor whole lung lavage lung transplant gene therapy rituximab gm-csf supplementation glucocorticoids Therapeutics. Pharmacology RM1-950 Iftikhar H Nair GB Kumar A Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis
description	Hira Iftikhar,1 Girish B Nair,1 Anupam Kumar2 1Division of Pulmonary and Critical Care, Beaumont Health, OUWB School of Medicine, Royal Oak, MI, USA; 2Division of Pulmonary and Critical Care, Baylor College of Medicine, Houston, TX, USACorrespondence: Hira Iftikhar Tel +1 2485251389Email hiraiftikhar@live.comAbstract: Pulmonary alveolar proteinosis (PAP) is a rare pulmonary surfactant homeostasis disorder resulting in buildup of lipo-proteinaceous material within the alveoli. PAP is classified as primary (autoimmune and hereditary), secondary, congenital and unclassifiable type based on the underlying pathogenesis. PAP has an insidious onset and can, in some cases, progress to severe respiratory failure. Diagnosis is often secured with bronchoalveolar lavage in the setting of classic imaging findings. Recent insights into genetic alterations and autoimmune mechanisms have provided newer diagnostics and treatment options. In this review, we discuss the etiopathogenesis, diagnosis and treatment options available and emerging for PAP.Keywords: alveolar macrophage, surfactant protein, granulocyte macrophage-colony stimulating factor, whole lung lavage, lung transplant, gene therapy, alveolar proteinosis
format	article
author	Iftikhar H Nair GB Kumar A
author_facet	Iftikhar H Nair GB Kumar A
author_sort	Iftikhar H
title	Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis
title_short	Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis
title_full	Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis
title_fullStr	Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis
title_full_unstemmed	Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis
title_sort	update on diagnosis and treatment of adult pulmonary alveolar proteinosis
publisher	Dove Medical Press
publishDate	2021
url	https://doaj.org/article/6b77820b22084e70bc52870c56b4bc87
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Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis

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