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Difference between revisions of "Sjögren syndrome"
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'''Sjögren syndrome''', also '''Sjögren disease''', is a disease that keeps rheumatologists busy. Sjögren is also spelled '''Sjoegren''' and '''Sjogren'''. | '''Sjögren syndrome''', also '''Sjögren disease''', is a disease that keeps [[rheumatology|rheumatologists]] busy. Sjögren is also spelled '''Sjoegren''' and '''Sjogren'''. | ||
The syndrome may be part of another [[connective tissue disorder]], e.g. [[rheumatoid arthritis]], in which case it is called ''secondary Sjögren syndrome''.<ref name=pmid1703737>{{Cite journal | last1 = Celenligil | first1 = H. | last2 = Kansu | first2 = E. | last3 = Ruacan | first3 = S. | last4 = Eratalay | first4 = K. | last5 = Irkeç | first5 = M. | title = Characterization of peripheral blood and salivary gland lymphocytes in secondary Sjögren's syndrome. | journal = Ann Dent | volume = 49 | issue = 2 | pages = 18-22 | month = | year = 1990 | doi = | PMID = 1703737 }}</ref> | |||
==General== | ==General== | ||
| Line 15: | Line 17: | ||
Features ([[salivary gland]]):<ref name=pmid19323360/> | Features ([[salivary gland]]):<ref name=pmid19323360/> | ||
*Lymphocytic infiltration - '''key feature'''. | *Lymphocytic infiltration - '''key feature'''. | ||
*+/- | *Viable [[salivary gland]] or [[lacrimal gland]] acini. | ||
*+/-[[Plasma cell]]s. | |||
*+/-Fibrosis. | *+/-Fibrosis. | ||
DDx: | DDx: | ||
*[[MALT lymphoma]]. | *[[MALT lymphoma]]. | ||
Note: | |||
*Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient. | |||
Images: | |||
*[http://img.medscape.com/pi/emed/ckb/rheumatology/329097-1339496-332125-1582482.jpg Sjögren syndrome (medscape.com)].<ref>URL: [http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa]. Accessed on: 24 July 2012.</ref> | |||
*[http://commons.wikimedia.org/wiki/File:Sjogren_syndrome_%281%29.jpg SS - low mag. (WC)]. | |||
*[http://commons.wikimedia.org/wiki/File:Sjogren_syndrome_%282%29.jpg SS - high mag. (WC)]. | |||
===Grading=== | |||
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> | |||
{| class="wikitable sortable" | |||
!Grade | |||
!Lymphocytes | |||
|- | |||
| 0 | |||
| absent | |||
|- | |||
| 1 | |||
| slight infiltrate | |||
|- | |||
| 2 | |||
| moderate infiltrate or less than one focus † | |||
|- | |||
| 3 | |||
| one focus † | |||
|- | |||
| 4 | |||
| more than one focus † | |||
|} | |||
† Focus = aggregrate of 50 lymphocytes. | |||
==See also== | ==See also== | ||
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==References== | ==References== | ||
{{Reflist| | {{Reflist|2}} | ||
[[Category:Clinical]] | [[Category:Clinical]] | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Syndromes]] | |||