|
|
Line 612: |
Line 612: |
| ==Pulmonary alveolar proteinosis== | | ==Pulmonary alveolar proteinosis== |
| *Abbreviated ''PAP''. | | *Abbreviated ''PAP''. |
| ===General===
| | {{Main|Pulmonary alveolar proteinosis}} |
| *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)].
| |
|
| |
|
| ==Diffuse panbronchiolitis== | | ==Diffuse panbronchiolitis== |