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Canadian Respiratory Journal
Volume 2016, Article ID 4064539, 4 pages
http://dx.doi.org/10.1155/2016/4064539
Case Report

Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory Failure

1Department of Internal Medicine, Queen’s University, Etherington Hall, Room 3033, 94 Stuart Street, Kingston, ON, Canada K7L 3N6
2Trillium Health Partners-Credit Valley Hospital Site, Mississauga, ON, Canada L5M 2N1
3University of Toronto, Trillium Health Partners-Credit Valley Hospital Site, Mississauga, ON, Canada L5M 2N1
4Laboratory Medicine and Genetics Program, Trillium Health Partners-Credit Valley Hospital Site, Mississauga, ON, Canada L5M 2N1

Received 4 December 2015; Revised 22 April 2016; Accepted 26 April 2016

Academic Editor: Alberto Ruano-Ravina

Copyright © 2016 Ryan R. Kroll et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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.