Difference between revisions of "Pulmonary alveolar proteinosis"

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Clinical:
Clinical:
*Dyspnea & cough - gradual onset.
*Dyspnea & cough - gradual onset.
*Anti-GM-CSF antibodies.<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=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><ref name=pmid16517574/>
*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>


===Radiology===
===Radiology===

Revision as of 18:47, 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.
  • Anti-GM-CSF antibodies - usually.[2]
  • Serum LDH elevated.[2][3]

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

www:

  • 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)

Images of DDx:

Stains

  • PAS +ve -- material in airspace (surfactant).[6]

IHC

  • Surfactant +ve.[7]

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. ↑ 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.
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