Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory Failure
Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by dysfunctional alveolar macrophages, which ineffectively clear surfactant and typically cause mild hypoxemia. Characteristic Computed Tomography findings are septal reticulations superimposed on ground-glass opacities in a craz...
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| Format: | Article |
| Language: | English |
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Wiley
2016-01-01
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| Series: | Canadian Respiratory Journal |
| Online Access: | http://dx.doi.org/10.1155/2016/4064539 |
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| author | Ryan R. Kroll Sameer Kumar Ronald F. Grossman Charles Price John R. Srigley |
| author_facet | Ryan R. Kroll Sameer Kumar Ronald F. Grossman Charles Price John R. Srigley |
| author_sort | Ryan R. Kroll |
| collection | DOAJ |
| description | Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by dysfunctional alveolar macrophages, which ineffectively clear surfactant and typically cause mild hypoxemia. Characteristic Computed Tomography findings are septal reticulations superimposed on ground-glass opacities in a crazy paving pattern, with a clear juxtaposition between affected and unaffected parenchyma. While traditionally PAP was diagnosed via biopsy, bronchoalveolar lavage (BAL) is usually sufficient; the fluid appears milky, and on microscopic examination there are foamy macrophages with eosinophilic granules and extracellular hyaline material that is Periodic Acid-Schiff positive. Standard therapy is whole lung lavage (WLL), although novel treatments are under development. The case presented is a 55-year-old woman with six months of progressive dyspnea, who developed hypoxemic respiratory failure requiring mechanical ventilation; she had typical findings of PAP on imaging and BAL. WLL was ultimately successful in restoring adequate oxygenation. Respiratory failure of this magnitude is a rare finding in PAP. |
| format | Article |
| id | doaj-art-d648df5bb3f54c30a8d5572c20c2f433 |
| institution | OA Journals |
| issn | 1198-2241 1916-7245 |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Canadian Respiratory Journal |
| spelling | doaj-art-d648df5bb3f54c30a8d5572c20c2f4332025-08-20T02:19:40ZengWileyCanadian Respiratory Journal1198-22411916-72452016-01-01201610.1155/2016/40645394064539Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory FailureRyan R. Kroll0Sameer Kumar1Ronald F. Grossman2Charles Price3John R. Srigley4Department of Internal Medicine, Queen’s University, Etherington Hall, Room 3033, 94 Stuart Street, Kingston, ON, K7L 3N6, CanadaTrillium Health Partners-Credit Valley Hospital Site, Mississauga, ON, L5M 2N1, CanadaUniversity of Toronto, Trillium Health Partners-Credit Valley Hospital Site, Mississauga, ON, L5M 2N1, CanadaTrillium Health Partners-Credit Valley Hospital Site, Mississauga, ON, L5M 2N1, CanadaLaboratory Medicine and Genetics Program, Trillium Health Partners-Credit Valley Hospital Site, Mississauga, ON, L5M 2N1, CanadaPulmonary alveolar proteinosis (PAP) is a rare condition characterized by dysfunctional alveolar macrophages, which ineffectively clear surfactant and typically cause mild hypoxemia. Characteristic Computed Tomography findings are septal reticulations superimposed on ground-glass opacities in a crazy paving pattern, with a clear juxtaposition between affected and unaffected parenchyma. While traditionally PAP was diagnosed via biopsy, bronchoalveolar lavage (BAL) is usually sufficient; the fluid appears milky, and on microscopic examination there are foamy macrophages with eosinophilic granules and extracellular hyaline material that is Periodic Acid-Schiff positive. Standard therapy is whole lung lavage (WLL), although novel treatments are under development. The case presented is a 55-year-old woman with six months of progressive dyspnea, who developed hypoxemic respiratory failure requiring mechanical ventilation; she had typical findings of PAP on imaging and BAL. WLL was ultimately successful in restoring adequate oxygenation. Respiratory failure of this magnitude is a rare finding in PAP.http://dx.doi.org/10.1155/2016/4064539 |
| spellingShingle | Ryan R. Kroll Sameer Kumar Ronald F. Grossman Charles Price John R. Srigley Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory Failure Canadian Respiratory Journal |
| title | Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory Failure |
| title_full | Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory Failure |
| title_fullStr | Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory Failure |
| title_full_unstemmed | Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory Failure |
| title_short | Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory Failure |
| title_sort | rare presentation of pulmonary alveolar proteinosis causing acute respiratory failure |
| url | http://dx.doi.org/10.1155/2016/4064539 |
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