Pulmonary alveolar proteinosis
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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]
- 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.
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]
- Pulmonary edema.
- Pneumocystis pneumonia - exudates foamy & vacuolated.
- Pulmonary hemorrhage (acute). (???)
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
- ↑ 1.0 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.
- ↑ Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 248. ISBN 978-0443066313.
- ↑ 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/.