Pulmonary Alveolar Proteinosis
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 predomin...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2012-01-01
|
| Series: | Canadian Respiratory Journal |
| Online Access: | http://dx.doi.org/10.1155/2012/841530 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832547021991444480 |
|---|---|
| author | Sandeep M Patel Hiroshi Sekiguchi Jordan P Reynolds Michael J Krowka |
| author_facet | Sandeep M Patel Hiroshi Sekiguchi Jordan P Reynolds Michael J Krowka |
| author_sort | Sandeep M Patel |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-886f6fcd18074f698d10ac0a5a286a9c |
| institution | Kabale University |
| issn | 1198-2241 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Canadian Respiratory Journal |
| spelling | doaj-art-886f6fcd18074f698d10ac0a5a286a9c2025-02-03T06:46:17ZengWileyCanadian Respiratory Journal1198-22412012-01-0119424324510.1155/2012/841530Pulmonary Alveolar ProteinosisSandeep M Patel0Hiroshi Sekiguchi1Jordan P Reynolds2Michael J Krowka3Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USADivision of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USADepartment of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USADivision of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USAPulmonary 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.http://dx.doi.org/10.1155/2012/841530 |
| spellingShingle | Sandeep M Patel Hiroshi Sekiguchi Jordan P Reynolds Michael J Krowka Pulmonary Alveolar Proteinosis Canadian Respiratory Journal |
| title | Pulmonary Alveolar Proteinosis |
| title_full | Pulmonary Alveolar Proteinosis |
| title_fullStr | Pulmonary Alveolar Proteinosis |
| title_full_unstemmed | Pulmonary Alveolar Proteinosis |
| title_short | Pulmonary Alveolar Proteinosis |
| title_sort | pulmonary alveolar proteinosis |
| url | http://dx.doi.org/10.1155/2012/841530 |
| work_keys_str_mv | AT sandeepmpatel pulmonaryalveolarproteinosis AT hiroshisekiguchi pulmonaryalveolarproteinosis AT jordanpreynolds pulmonaryalveolarproteinosis AT michaeljkrowka pulmonaryalveolarproteinosis |