Difference between revisions of "Pulmonary alveolar proteinosis"

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| 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
| Stains    = PAS +ve, PASD +ve
| IHC        = surfactant +ve
| IHC        = surfactant +ve
| EM        = lamellar bodies
| EM        = lamellar bodies
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| 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.
#*Abnormal surfactant.
#**GM-CSF receptor defect.
#*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" - see: [http://radiographics.rsnajnls.org/cgi/content/figsonly/23/6/1509 http://radiographics.rsnajnls.org/cgi/content/figsonly/23/6/1509].
*HRCT: "[[crazy paving]]".


==Microscopic==
==Microscopic==
Line 69: Line 77:
*[[Pneumocystis pneumonia]] - exudates foamy & vacuolated.
*[[Pneumocystis pneumonia]] - exudates foamy & vacuolated.
*Pulmonary hemorrhage (acute). (???)
*Pulmonary hemorrhage (acute). (???)
*Mycobacterial infection.
*Nocardia infection.


===Images===
===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>
<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)
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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 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>
*[[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 (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==

Latest revision as of 17:22, 4 November 2021

Pulmonary alveolar proteinosis
Diagnosis in short

Pulmonary alveolar proteinosis. H&E stain.
LM "dense bodies" or "chatter" (represent dead macrophages) within acellular eosinophilic material that is in the alveoli
LM DDx pulmonary edema, pneumocystis pneumonia, pulmonary hemorrhage (acute), mycobacterial infection, Nocardia infection
Stains PAS +ve, PASD +ve
IHC surfactant +ve
EM lamellar bodies
Site lung - see medical lung diseases
Associated Dx +/-hematologic malignancy
Clinical history +/-infection, +/-smoking
Signs decreased oxygen saturation
Prevalence uncommon
Blood work anti-GM-CSF antibodies, LDH elevated
Radiology airspace disease, "crazy paving"
Prognosis benign

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.
  • Anti-GM-CSF antibodies - usually.[2]
  • Serum LDH elevated.[2][3]
  • Decreased oxygen saturation.

Gross

Chest CT showing crazy paving pattern. (WC/Verma)
  • Consolidation.

  • PAP. (WC/Rosen)

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:[4]

Images

  • PAP - low mag. (WC)

  • PAP - intermed. mag. (WC)

  • PAP - high mag. (WC)

  • PAP - very high mag. (WC)

  • PAP - intermed. mag. (WC)

  • PAP - very high mag. (WC)

  • PAP (WC/Rosen)

  • PAP (WC/Rosen)

www:

Images of DDx (pulmonary edema):

Stains

IHC

EM

  • Lamellar bodies.[13]

Note:

  • Lamellar bodies are also seen in secreting adenocarcinoma, obstructive pneumonitis.

Sign out

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.

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

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. ↑ 2.0 2.1 Lin, FC.; Chang, GD.; Chern, MS.; Chen, YC.; Chang, SC. (Jun 2006). "Clinical significance of anti-GM-CSF antibodies in idiopathic pulmonary alveolar proteinosis.". Thorax 61 (6): 528-34. doi:10.1136/thx.2005.054171. PMID 16517574.
  3. ↑ Bhattacharyya, D.; Barthwal, MS.; Katoch, CD.; Rohatgi, MG.; Hasnain, S.; Rai, SP.; Arora, A. (Jan 2013). "Primary alveolar proteinosis - A report of two cases.". Med J Armed Forces India 69 (1): 90-3. doi:10.1016/j.mjafi.2012.02.016. PMID 24532945.
  4. ↑ Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 248. ISBN 978-0443066313.
  5. ↑ 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/.
  6. ↑ Ben-Dov, I.; Segel, MJ. (Jan 2014). "Autoimmune pulmonary alveolar proteinosis: Clinical course and diagnostic criteria.". Autoimmun Rev. doi:10.1016/j.autrev.2014.01.046. PMID 24424195.
  7. ↑ Mikami, T.; Yamamoto, Y.; Yokoyama, M.; Okayasu, I. (Dec 1997). "Pulmonary alveolar proteinosis: diagnosis using routinely processed smears of bronchoalveolar lavage fluid.". J Clin Pathol 50 (12): 981-4. PMID 9516877.
  8. ↑ Maygarden, SJ.; Iacocca, MV.; Funkhouser, WK.; Novotny, DB. (Jun 2001). "Pulmonary alveolar proteinosis: a spectrum of cytologic, histochemical, and ultrastructural findings in bronchoalveolar lavage fluid.". Diagn Cytopathol 24 (6): 389-95. PMID 11391819.
  9. ↑ Meng, ZL.; Liu, HR.; Liang, ZY.; Zhang, SY. (Sep 2005). "[Pathologic feature and diagnosis of pulmonary alveolar proteinosis].". Zhonghua Bing Li Xue Za Zhi 34 (9): 575-8. PMID 16468308.
  10. ↑ URL: http://pathhsw5m54.ucsf.edu/overview/fungi1a.html. Accessed on: 3 March 2014.
  11. ↑ Hwang DM. April 1, 2014.
  12. ↑ Albores, J.; Seki, A.; Fishbein, MC.; Abtin, F.; Lynch, JP.; Wang, T.; Weigt, SS. (Jun 2013). "A rare occurrence of pulmonary alveolar proteinosis after lung transplantation.". Semin Respir Crit Care Med 34 (3): 431-8. doi:10.1055/s-0033-1348472. PMID 23821516.
  13. ↑ Luo, BF.; Li, HP.; Yi, XH.; Tao, JW.; Lü, HJ.; Fang, X.; Zhang, ZM.; Zhang, L. et al. (Dec 2012). "[The ultrastructural features of sputum deposition and its value in the diagnosis of pulmonary alveolar proteinosis].". Zhonghua Jie He He Hu Xi Za Zhi 35 (12): 887-91. PMID 23328177.

External links

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