Difference between revisions of "Pulmonary alveolar proteinosis"

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#redirect [[Medical_lung_diseases#Pulmonary_alveolar_proteinosis]]
'''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]]

Revision as of 18:20, 26 February 2014

Pulmonary_alveolar_proteinosis, abbreviated PAP, in an uncommon medical lung disease.

General

  • Associated with smoking - particularily in men.[1]

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:[1]

  1. Congenital:
      • Abnormal surfactant.
      • GM-CSF receptor defect.
  2. Secondary:
    • Infections.
    • Haematologic malignancy.
  3. Acquired:
    • Dusts - interfere with macrophage function.

Clinical:

  • Dyspnea & cough - gradual onset.

Radiology

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:[2]

Images

www:

  • PAP - low mag. (WC)

  • PAP - high mag. (WC)

  • PAP - intermed. mag. (WC)

  • PAP - very high mag. (WC)

Images of DDx:

See also

References

  1. ↑ 1.0 1.1 Trapnell BC, Whitsett JA, Nakata K (December 2003). "Pulmonary alveolar proteinosis". N. Engl. J. Med. 349 (26): 2527-39. doi:10.1056/NEJMra023226. PMID 14695413. http://content.nejm.org/cgi/content/extract/349/26/2527.
  2. ↑ Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 248. ISBN 978-0443066313.
  3. ↑ Leslie KO (May 2009). "My approach to interstitial lung disease using clinical, radiological and histopathological patterns". J. Clin. Pathol. 62 (5): 387–401. doi:10.1136/jcp.2008.059782. PMC 2668105. PMID 19398592. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2668105/.
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