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Pulmonary alveolar proteinosis (view source)
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# | '''Pulmonary_alveolar_proteinosis''', abbreviated '''PAP''', in an uncommon [[medical lung diseases|medical lung disease]]. | ||
==General== | |||
*Associated with smoking - particularily in men.<ref name=pmid14695413>{{cite journal |author=Trapnell BC, Whitsett JA, Nakata K |title=Pulmonary alveolar proteinosis |journal=N. Engl. J. Med. |volume=349 |issue=26 |pages=2527-39 |year=2003 |month=December |pmid=14695413 |doi=10.1056/NEJMra023226 |url=http://content.nejm.org/cgi/content/extract/349/26/2527}}</ref> | |||
Pathophysiology: | |||
*GM-CSF (granulocyte-macrophage colony stimulating factor) signaling in macrophages/lack of GM-CSF. | |||
**GM-CSF is required by alveolar macrophages to clear surfactant. | |||
Classification:<ref name=pmid14695413/> | |||
#Congenital: | |||
#**Abnormal surfactant. | |||
#**GM-CSF receptor defect. | |||
#Secondary: | |||
#*Infections. | |||
#*Haematologic malignancy. | |||
#Acquired: | |||
#*Dusts - interfere with macrophage function. | |||
Clinical: | |||
*Dyspnea & cough - gradual onset. | |||
===Radiology=== | |||
*CXR: airspace disease. | |||
*HRCT: "crazy paving" - see: [http://radiographics.rsnajnls.org/cgi/content/figsonly/23/6/1509 http://radiographics.rsnajnls.org/cgi/content/figsonly/23/6/1509]. | |||
==Microscopic== | |||
Features: | |||
*Crap in the alveoli: | |||
*"Dense bodies" - dead macrophages ("Chatter" in the alveoli). | |||
**Edema - has pink stuff in the alveoli like PAP but no ''dense bodies''. | |||
DDx - may mimic:<ref>{{Ref PPP|248}}</ref> | |||
*[[Pulmonary edema]]. | |||
*[[Pneumocystis pneumonia]] - exudates foamy & vacuolated. | |||
*Pulmonary hemorrhage (acute). (???) | |||
===Images=== | |||
www: | |||
*[http://jcp.bmjjournals.com/content/62/5/387/F23.large.jpg PAP (bmjjournals.com)].<ref name=pmid19398592>{{cite journal |author=Leslie KO |title=My approach to interstitial lung disease using clinical, radiological and histopathological patterns |journal=J. Clin. Pathol. |volume=62 |issue=5 |pages=387–401 |year=2009 |month=May |pmid=19398592 |pmc=2668105 |doi=10.1136/jcp.2008.059782 |url=}}</ref> | |||
<gallery> | |||
Image:Pulmonary_alveolar_proteinosis_-3-_low_mag.jpg | PAP - low mag. (WC) | |||
Image:Pulmonary_alveolar_proteinosis_-3-_high_mag.jpg | PAP - high mag. (WC) | |||
</gallery> | |||
<gallery> | |||
Image:Pulmonary_alveolar_proteinosis_-_2_-_intermed_mag.jpg | PAP - intermed. mag. (WC) | |||
Image:Pulmonary_alveolar_proteinosis_-_very_high_mag.jpg | PAP - very high mag. (WC) | |||
</gallery> | |||
Images of DDx: | |||
*[http://www.sciencephoto.com/media/98443/enlarge Pulmonary edema (sciencephoto.com)]. | |||
==See also== | |||
*[[Medical lung diseases]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Medical lung diseases]] | |||