Difference between revisions of "Sjögren syndrome"

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==Microscopic==
==Microscopic==
Features ([[salivary gland]]):<ref name=pmid19323360/>
Features ([[salivary gland]]):<ref name=pmid19323360/>
*Lymphocytic infiltration - '''key feature'''.
*Viable [[salivary gland]] (or [[lacrimal gland]]) acini with significant lymphocytic infiltration - '''key feature'''.
**"Benign lymphoepithelial lesion"<ref name=pmid15956090/> - intraepithelial lymphocytes.
**Plasma cells should not exceed 10% of the inflammatory infiltrate.<ref name=pmid12022353/>
*Viable [[salivary gland]] or [[lacrimal gland]] acini.
**"Significant" infiltrate = cluster of >= 50 lymphocytes - '''important'''.
*+/-[[Plasma cell]]s.
***Lymphocytes that are perivascular count.<ref name=pmid12022353/>
***May have "benign lymphoepithelial lesion"<ref name=pmid15956090/> - intraepithelial lymphocytes.
*+/-Fibrosis.
*+/-Fibrosis.


DDx:
DDx:
*[[MALT lymphoma]].
*[[MALT lymphoma]].
*[[Chronic sialadenitis]].


Note:
Note:
*Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient.
*Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient.
*Perivascular lymphocytes ''not'' important.


Images:
Images:
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*[http://commons.wikimedia.org/wiki/File:Sjogren_syndrome_%282%29.jpg SS - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Sjogren_syndrome_%282%29.jpg SS - high mag. (WC)].


===Grading===
===Focus score===
Lesions can be graded with the ''Chisholm-Mason classification''.<ref name=pmid15956090>{{Cite journal  | last1 = Ramos-Casals | first1 = M. | last2 = Font | first2 = J. | title = Primary Sjögren's syndrome: current and emergent aetiopathogenic concepts. | journal = Rheumatology (Oxford) | volume = 44 | issue = 11 | pages = 1354-67 | month = Nov | year = 2005 | doi = 10.1093/rheumatology/keh714 | PMID = 15956090 | url = http://rheumatology.oxfordjournals.org/content/44/11/1354.long }}</ref> It is based on assessing 4 mm<sup>2</sup> area of salivary gland tissue and depends on the abundance and aggregation of lymphocytes as follows:<ref>{{Cite journal  | last1 = Chisholm | first1 = DM. | last2 = Mason | first2 = DK. | title = Labial salivary gland biopsy in Sjögren's disease. | journal = J Clin Pathol | volume = 21 | issue = 5 | pages = 656-60 | month = Sep | year = 1968 | doi =  | PMID = 5697370 | PMC = 473887 | url = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC473887/?tool=pubmed }}</ref>
*This is a count of significant lymphocytic foci.
 
====Grading - historical====
In the past lesions were graded with the ''Chisholm-Mason classification''.<ref name=pmid15956090>{{Cite journal  | last1 = Ramos-Casals | first1 = M. | last2 = Font | first2 = J. | title = Primary Sjögren's syndrome: current and emergent aetiopathogenic concepts. | journal = Rheumatology (Oxford) | volume = 44 | issue = 11 | pages = 1354-67 | month = Nov | year = 2005 | doi = 10.1093/rheumatology/keh714 | PMID = 15956090 | url = http://rheumatology.oxfordjournals.org/content/44/11/1354.long }}</ref>  
 
It is based on assessing 4 mm<sup>2</sup> area of salivary gland tissue and depends on the abundance and aggregation of lymphocytes as follows:<ref>{{Cite journal  | last1 = Chisholm | first1 = DM. | last2 = Mason | first2 = DK. | title = Labial salivary gland biopsy in Sjögren's disease. | journal = J Clin Pathol | volume = 21 | issue = 5 | pages = 656-60 | month = Sep | year = 1968 | doi =  | PMID = 5697370 | PMC = 473887 | url = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC473887/?tool=pubmed }}</ref>
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{| class="wikitable sortable"  
!Grade  
!Grade  
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