Pulmonary alveolar proteinosis: Difference between revisions
Jump to navigation
Jump to search
tweak |
|||
| (11 intermediate revisions by the same user not shown) | |||
| 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 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 73: | Line 81: | ||
===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 85: | 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 92: | 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== | ||
| Line 111: | Line 125: | ||
</pre> | </pre> | ||
===Micro=== | ====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. | 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. | ||
===Cytology=== | |||
In conjunction with history and imaging, PAP may be suggested on cytology.<ref name=pmid29607238>{{cite journal |authors=Li M, Alowami S, Schell M, Davis C, Naqvi A |title=Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse |journal=Case Rep Pulmonol |volume=2018 |issue= |pages=5202173 |date=2018 |pmid=29607238 |pmc=5828087 |doi=10.1155/2018/5202173 |url=}}</ref> | |||
<pre> | |||
Right Lung, BAL: NEGATIVE for Malignancy. Macrophages and amorphous material present in a dirty background, see comment. | |||
Comment: | |||
Satisfactory for evaluation. | |||
PAS POSITIVE and PSAD POSITIVE staining globules are present. GMS stain for pneumocystis is NEGATIVE. | |||
In the proper clinical context, the findings may be in keeping with pulmonary alveolar proteinosis (PAP). | |||
In the context of possible PAP, GM-CSF testing is suggested, if not already done. Please correlate with clinical and radiologic findings. | |||
The case was partially reviewed internally. | |||
</pre> | |||
==See also== | ==See also== | ||