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Pulmonary alveolar proteinosis (view source)
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Pulmonary_alveolar_proteinosis_-3-_intermed_mag.jpg | |||
| Width = | |||
| Caption = Pulmonary alveolar proteinosis. [[H&E stain]]. | |||
| Synonyms = | |||
| Micro = "dense bodies" or "chatter" (represent dead macrophages) within acellular eosinophilic material that is in the alveoli | |||
| Subtypes = | |||
| LMDDx = [[pulmonary edema]], [[pneumocystis pneumonia]], [[pulmonary hemorrhage]] (acute), mycobacterial infection, Nocardia infection | |||
| Stains = PAS +ve, PASD +ve | |||
| IHC = surfactant +ve | |||
| EM = lamellar bodies | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[lung]] - see ''[[medical lung diseases]]'' | |||
| Assdx = +/-hematologic malignancy | |||
| Syndromes = | |||
| Clinicalhx = +/-infection, +/-[[smoking]] | |||
| Signs = decreased oxygen saturation | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = anti-GM-CSF antibodies, LDH elevated | |||
| Rads = airspace disease, "[[crazy paving]]" | |||
| Endoscopy = | |||
| Prognosis = benign | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''Pulmonary alveolar proteinosis''', abbreviated '''PAP''', in an uncommon [[medical lung diseases|medical lung disease]]. | '''Pulmonary alveolar proteinosis''', abbreviated '''PAP''', in an uncommon [[medical lung diseases|medical lung disease]]. | ||
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Classification:<ref name=pmid14695413/> | Classification:<ref name=pmid14695413/> | ||
#Congenital: | #Congenital: | ||
# | #*Abnormal surfactant. | ||
# | #*GM-CSF receptor defect. | ||
#Secondary: | #Secondary: | ||
#*Infections. | #*Infections. | ||
| Line 19: | Line 50: | ||
Clinical: | Clinical: | ||
*Dyspnea & cough - gradual onset. | *[[Dyspnea]] & cough - gradual onset. | ||
*Anti-GM-CSF antibodies - usually.<ref name=pmid16517574>{{Cite journal | last1 = Lin | first1 = FC. | last2 = Chang | first2 = GD. | last3 = Chern | first3 = MS. | last4 = Chen | first4 = YC. | last5 = Chang | first5 = SC. | title = Clinical significance of anti-GM-CSF antibodies in idiopathic pulmonary alveolar proteinosis. | journal = Thorax | volume = 61 | issue = 6 | pages = 528-34 | month = Jun | year = 2006 | doi = 10.1136/thx.2005.054171 | PMID = 16517574 }}</ref> | |||
*Serum LDH elevated.<ref name=pmid16517574/><ref name=pmid24532945>{{Cite journal | last1 = Bhattacharyya | first1 = D. | last2 = Barthwal | first2 = MS. | last3 = Katoch | first3 = CD. | last4 = Rohatgi | first4 = MG. | last5 = Hasnain | first5 = S. | last6 = Rai | first6 = SP. | last7 = Arora | first7 = A. | title = Primary alveolar proteinosis - A report of two cases. | journal = Med J Armed Forces India | volume = 69 | issue = 1 | pages = 90-3 | month = Jan | year = 2013 | doi = 10.1016/j.mjafi.2012.02.016 | PMID = 24532945 }}</ref> | |||
*Decreased oxygen saturation. | |||
==Gross== | |||
[[File:Crazy paving pattern on chest CT scan.jpg|thumb|Chest CT showing [[crazy paving]] pattern. (WC/Verma)]] | |||
*Consolidation. | |||
<gallery> | |||
Image:Alveolar proteinosis (5397198260).jpg | PAP. (WC/Rosen) | |||
</gallery> | |||
===Radiology=== | ===Radiology=== | ||
*CXR: airspace disease. | *CXR: airspace disease. | ||
*HRCT: "crazy paving" | *HRCT: "[[crazy paving]]". | ||
==Microscopic== | ==Microscopic== | ||
| Line 35: | Line 77: | ||
*[[Pneumocystis pneumonia]] - exudates foamy & vacuolated. | *[[Pneumocystis pneumonia]] - exudates foamy & vacuolated. | ||
*Pulmonary hemorrhage (acute). (???) | *Pulmonary hemorrhage (acute). (???) | ||
*Mycobacterial infection. | |||
*Nocardia infection. | |||
===Images=== | ===Images=== | ||
<gallery> | <gallery> | ||
Image:Pulmonary_alveolar_proteinosis_-3-_low_mag.jpg | PAP - low mag. (WC) | Image:Pulmonary_alveolar_proteinosis_-3-_low_mag.jpg | PAP - low mag. (WC) | ||
Image:Pulmonary_alveolar_proteinosis_-3-_intermed_mag.jpg | PAP - intermed. mag. (WC) | |||
Image:Pulmonary_alveolar_proteinosis_-3-_high_mag.jpg | PAP - high mag. (WC) | Image:Pulmonary_alveolar_proteinosis_-3-_high_mag.jpg | PAP - high mag. (WC) | ||
Image:Pulmonary_alveolar_proteinosis_-3-_very high_mag.jpg | PAP - very high mag. (WC) | |||
</gallery> | </gallery> | ||
<gallery> | <gallery> | ||
| Line 47: | Line 91: | ||
Image:Pulmonary_alveolar_proteinosis_-_very_high_mag.jpg | PAP - very high mag. (WC) | Image:Pulmonary_alveolar_proteinosis_-_very_high_mag.jpg | PAP - very high mag. (WC) | ||
</gallery> | </gallery> | ||
Images of DDx: | <gallery> | ||
Image:Alveolar proteinosis (5396599841).jpg | PAP (WC/Rosen) | |||
Image:Alveolar proteinosis (5397198452).jpg | PAP (WC/Rosen) | |||
</gallery> | |||
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> | |||
Images of DDx (pulmonary edema): | |||
*[http://www.sciencephoto.com/media/98443/enlarge Pulmonary edema (sciencephoto.com)]. | *[http://www.sciencephoto.com/media/98443/enlarge Pulmonary edema (sciencephoto.com)]. | ||
==Stains== | ==Stains== | ||
*PAS +ve -- material in airspace (surfactant).<ref name=pmid24424195>{{Cite journal | last1 = Ben-Dov | first1 = I. | last2 = Segel | first2 = MJ. | title = Autoimmune pulmonary alveolar proteinosis: Clinical course and diagnostic criteria. | journal = Autoimmun Rev | volume = | issue = | pages = | month = Jan | year = 2014 | doi = 10.1016/j.autrev.2014.01.046 | PMID = 24424195 }}</ref> | *[[PAS stain|PAS]] +ve -- material in airspace (surfactant).<ref name=pmid24424195>{{Cite journal | last1 = Ben-Dov | first1 = I. | last2 = Segel | first2 = MJ. | title = Autoimmune pulmonary alveolar proteinosis: Clinical course and diagnostic criteria. | journal = Autoimmun Rev | volume = | issue = | pages = | month = Jan | year = 2014 | doi = 10.1016/j.autrev.2014.01.046 | PMID = 24424195 }}</ref><ref>{{Cite journal | last1 = Mikami | first1 = T. | last2 = Yamamoto | first2 = Y. | last3 = Yokoyama | first3 = M. | last4 = Okayasu | first4 = I. | title = Pulmonary alveolar proteinosis: diagnosis using routinely processed smears of bronchoalveolar lavage fluid. | journal = J Clin Pathol | volume = 50 | issue = 12 | pages = 981-4 | month = Dec | year = 1997 | doi = | PMID = 9516877 }}</ref> | ||
*[[PASD stain|PASD]] +ve<ref>{{Cite journal | last1 = Maygarden | first1 = SJ. | last2 = Iacocca | first2 = MV. | last3 = Funkhouser | first3 = WK. | last4 = Novotny | first4 = DB. | title = Pulmonary alveolar proteinosis: a spectrum of cytologic, histochemical, and ultrastructural findings in bronchoalveolar lavage fluid. | journal = Diagn Cytopathol | volume = 24 | issue = 6 | pages = 389-95 | month = Jun | year = 2001 | doi = | PMID = 11391819 }}</ref> (red<ref name=pmid16468308>{{Cite journal | last1 = Meng | first1 = ZL. | last2 = Liu | first2 = HR. | last3 = Liang | first3 = ZY. | last4 = Zhang | first4 = SY. | title = [Pathologic feature and diagnosis of pulmonary alveolar proteinosis]. | journal = Zhonghua Bing Li Xue Za Zhi | volume = 34 | issue = 9 | pages = 575-8 | month = Sep | year = 2005 | doi = | PMID = 16468308 }}</ref>). | |||
**[[Pulmonary edema]] fluid -ve.<ref>URL: [http://pathhsw5m54.ucsf.edu/overview/fungi1a.html http://pathhsw5m54.ucsf.edu/overview/fungi1a.html]. Accessed on: 3 March 2014.</ref> | |||
**Occasionally, PAP may be PASD -ve.<ref>Hwang DM. April 1, 2014.</ref> | |||
==IHC== | ==IHC== | ||
*Surfactant +ve.<ref name=pmid23821516>{{Cite journal | last1 = Albores | first1 = J. | last2 = Seki | first2 = A. | last3 = Fishbein | first3 = MC. | last4 = Abtin | first4 = F. | last5 = Lynch | first5 = JP. | last6 = Wang | first6 = T. | last7 = Weigt | first7 = SS. | title = A rare occurrence of pulmonary alveolar proteinosis after lung transplantation. | journal = Semin Respir Crit Care Med | volume = 34 | issue = 3 | pages = 431-8 | month = Jun | year = 2013 | doi = 10.1055/s-0033-1348472 | PMID = 23821516 }}</ref> | *Surfactant +ve.<ref name=pmid23821516>{{Cite journal | last1 = Albores | first1 = J. | last2 = Seki | first2 = A. | last3 = Fishbein | first3 = MC. | last4 = Abtin | first4 = F. | last5 = Lynch | first5 = JP. | last6 = Wang | first6 = T. | last7 = Weigt | first7 = SS. | title = A rare occurrence of pulmonary alveolar proteinosis after lung transplantation. | journal = Semin Respir Crit Care Med | volume = 34 | issue = 3 | pages = 431-8 | month = Jun | year = 2013 | doi = 10.1055/s-0033-1348472 | PMID = 23821516 }}</ref> | ||
==EM== | |||
*Lamellar bodies.<ref>{{Cite journal | last1 = Luo | first1 = BF. | last2 = Li | first2 = HP. | last3 = Yi | first3 = XH. | last4 = Tao | first4 = JW. | last5 = Lü | first5 = HJ. | last6 = Fang | first6 = X. | last7 = Zhang | first7 = ZM. | last8 = Zhang | first8 = L. | last9 = Ren | first9 = SX. | title = [The ultrastructural features of sputum deposition and its value in the diagnosis of pulmonary alveolar proteinosis]. | journal = Zhonghua Jie He He Hu Xi Za Zhi | volume = 35 | issue = 12 | pages = 887-91 | month = Dec | year = 2012 | doi = | PMID = 23328177 }}</ref> | |||
Note: | |||
*Lamellar bodies are also seen in secreting adenocarcinoma, obstructive pneumonitis. | |||
==Sign out== | |||
<pre> | |||
LUNG, RIGHT UPPER LOBE (ABNORMAL AREA ON CT), WEDGE RESECTION: | |||
- PULMONARY ALVEOLAR PROTEINOSIS, SEE COMMENT. | |||
- NO SIGNIFICANT INTERSTITIAL FIBROSIS. | |||
- NO SIGNIFICANT INFLAMMATION. | |||
- NEGATIVE FOR MALIGNANCY. | |||
</pre> | |||
===Micro=== | |||
Sections show lung with eosinophilic material in the airspaces. Focally, small (~20 micrometres), more dense appearing, bodies are also in the airspace. The alveolar walls are within normal limits. No significant inflammation is identified. No microorganisms are seen with routine stains. There is no pulmonary hemorrhage. | |||
==See also== | ==See also== | ||
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==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
==External links== | |||
*[http://pathhsw5m54.ucsf.edu/overview/fungi1a.html DDx of pink staining on lung (ucsf.edu)]. | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Medical lung diseases]] | [[Category:Medical lung diseases]] | ||