Difference between revisions of "Sjögren syndrome"
Jump to navigation
Jump to search
(→References: +diagnosis) |
(nl update 24) |
||
| Line 1: | Line 1: | ||
'''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== | ||
| Line 27: | Line 61: | ||
==References== | ==References== | ||
{{Reflist| | {{Reflist|2}} | ||
[[Category:Clinical]] | [[Category:Clinical]] | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Syndromes]] | |||
Revision as of 01:51, 10 August 2012
Sjögren syndrome, also Sjögren disease, is a disease that keeps 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.[1]
General
Clinical - classically:
- Women in 50s.
- Dry mouth (xerostomia).
- Dry eyes (xerophthalmia).
Blood work:[2]
- ANA +ve.
- Anti-SSA (Ro) +ve.
- Anti-SSB (La) +ve.
Microscopic
Features (salivary gland):[2]
- Lymphocytic infiltration - key feature.
- Viable salivary gland or lacrimal gland acini.
- +/-Plasma cells.
- +/-Fibrosis.
DDx:
Note:
- Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient.
Images:
Grading
Lesions can be graded with the Chisholm-Mason classification.[4] It is based on assessing 4 mm2 area of salivary gland tissue and depends on the abundance and aggregation of lymphocytes as follows:[5]
| 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
References
- ↑ Celenligil, H.; Kansu, E.; Ruacan, S.; Eratalay, K.; Irkeç, M. (1990). "Characterization of peripheral blood and salivary gland lymphocytes in secondary Sjögren's syndrome.". Ann Dent 49 (2): 18-22. PMID 1703737.
- ↑ 2.0 2.1 "Information from your family doctor. Sjögren syndrome.". Am Fam Physician 79 (6): 472. Mar 2009. PMID 19323360.
- ↑ URL: http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa. Accessed on: 24 July 2012.
- ↑ Ramos-Casals, M.; Font, J. (Nov 2005). "Primary Sjögren's syndrome: current and emergent aetiopathogenic concepts.". Rheumatology (Oxford) 44 (11): 1354-67. doi:10.1093/rheumatology/keh714. PMID 15956090. http://rheumatology.oxfordjournals.org/content/44/11/1354.long.
- ↑ Chisholm, DM.; Mason, DK. (Sep 1968). "Labial salivary gland biopsy in Sjögren's disease.". J Clin Pathol 21 (5): 656-60. PMC 473887. PMID 5697370. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC473887/?tool=pubmed.