Document Detail


Pulmonary alveolar proteinosis.
MedLine Citation:
PMID:  22891182     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Pulmonary alveolar proteinosis (PAP) is a disease of alveolar accumulation of phospholipoproteinaceous material that results in gas exchange impairment leading to dyspnea and alveolar infiltrates. There are three forms of PAP: congenital, acquired and idiopathic; of which the latter two are predominant in the adult population. Previous case studies have found that the acquired form can be secondary to various autoimmune, infectious, malignant and environmental etiologies. Recent advances in the understanding of the pathophysiology of PAP demonstrate that the idiopathic form is due to antigranulocyte macrophage-colony stimulating factor antibodies. Therapeutic targets that replace granulocyte macrophage colony stimulating factor or remove these antibodies are being actively developed. The current standard of care is to perform whole lung lavage on these patients to clear the alveolar space to help improve respiratory physiology. A case of PAP is reported, followed by a literature review on the diagnosis and management of this rare condition with the aim of increasing awareness among physicians when treating patients who present with alveolar infiltrates.
Authors:
Sandeep M Patel; Hiroshi Sekiguchi; Jordan P Reynolds; Michael J Krowka
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Canadian respiratory journal : journal of the Canadian Thoracic Society     Volume:  19     ISSN:  1916-7245     ISO Abbreviation:  Can. Respir. J.     Publication Date:    2012 Jul-Aug
Date Detail:
Created Date:  2012-08-14     Completed Date:  2013-04-09     Revised Date:  2013-07-12    
Medline Journal Info:
Nlm Unique ID:  9433332     Medline TA:  Can Respir J     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  243-5     Citation Subset:  IM    
Affiliation:
Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
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MeSH Terms
Descriptor/Qualifier:
Adult
Autoantibodies / immunology
Bronchoalveolar Lavage
Granulocyte-Macrophage Colony-Stimulating Factor / immunology,  therapeutic use
Humans
Male
Pulmonary Alveolar Proteinosis* / diagnosis,  physiopathology,  therapy
Chemical
Reg. No./Substance:
0/Autoantibodies; 83869-56-1/Granulocyte-Macrophage Colony-Stimulating Factor
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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