49,012
edits
Difference between revisions of "Pulmonary alveolar proteinosis"
Jump to navigation
Jump to search
Pulmonary alveolar proteinosis (view source)
Revision as of 17:22, 4 November 2021
, 17:22, 4 November 2021no edit summary
| (9 intermediate revisions by the same user not shown) | |||
| Line 7: | Line 7: | ||
| Micro = "dense bodies" or "chatter" (represent dead macrophages) within acellular eosinophilic material that is in the alveoli | | Micro = "dense bodies" or "chatter" (represent dead macrophages) within acellular eosinophilic material that is in the alveoli | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[pulmonary edema]], [[pneumocystis pneumonia]], [[pulmonary hemorrhage]] (acute) | | LMDDx = [[pulmonary edema]], [[pneumocystis pneumonia]], [[pulmonary hemorrhage]] (acute), mycobacterial infection, Nocardia infection | ||
| Stains = PAS +ve, PASD +ve | | Stains = PAS +ve, PASD +ve | ||
| IHC = surfactant +ve | | IHC = surfactant +ve | ||
| Line 23: | Line 23: | ||
| Prevalence = uncommon | | Prevalence = uncommon | ||
| Bloodwork = anti-GM-CSF antibodies, LDH elevated | | Bloodwork = anti-GM-CSF antibodies, LDH elevated | ||
| Rads = airspace disease, "crazy paving" | | Rads = airspace disease, "[[crazy paving]]" | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = benign | | Prognosis = benign | ||
| Line 41: | Line 41: | ||
Classification:<ref name=pmid14695413/> | Classification:<ref name=pmid14695413/> | ||
#Congenital: | #Congenital: | ||
# | #*Abnormal surfactant. | ||
# | #*GM-CSF receptor defect. | ||
#Secondary: | #Secondary: | ||
#*Infections. | #*Infections. | ||
| Line 50: | 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> | *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> | *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. | *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 70: | Line 78: | ||
*Pulmonary hemorrhage (acute). (???) | *Pulmonary hemorrhage (acute). (???) | ||
*Mycobacterial infection. | *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) | ||
| Line 84: | 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)]. | ||
| Line 91: | Line 105: | ||
*[[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>). | *[[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> | **[[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== | ||