Difference between revisions of "Sjögren syndrome"
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==Microscopic== | ==Microscopic== | ||
Features ([[salivary gland]]):<ref name=pmid19323360/> | Features ([[salivary gland]]):<ref name=pmid19323360/> | ||
* | *Viable [[salivary gland]] (or [[lacrimal gland]]) acini with significant lymphocytic infiltration - '''key feature'''. | ||
** | **Plasma cells should not exceed 10% of the inflammatory infiltrate.<ref name=pmid12022353/> | ||
* | **"Significant" infiltrate = cluster of >= 50 lymphocytes - '''important'''. | ||
* | ***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. | ||
Images: | Images: | ||
| Line 68: | Line 69: | ||
*[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=== | ||
*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> | |||
{| class="wikitable sortable" | {| class="wikitable sortable" | ||
!Grade | !Grade | ||
Revision as of 15:10, 11 April 2013
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).
Diagnostic criteria
European criteria of 2002:[2]
| Criteria | Details | Type |
|---|---|---|
| Oral symptoms | any: (1) dry mouth > 3 months, (2) require fluids for swallowing, (3) swollen salivary glands (adults) | history |
| Oral signs | any: (1) low salivary flow test positive, (2) salivary scintigraphy positive (3) (parotid) sialography positive | clinical test |
| Ocular symptoms | any: (1) dry eye > 3 months, (2) need artifical tears >3x/day, (3) sand or gravel in the eyes sensation | history |
| Ocular signs | any: (1) Schirmer's test positive, (2) ocular dye test positive | clinical test |
| Autoantibodies | anti-SSA/Ro and/or anti-SSB/La | serology |
| Histology | labial minor salivary gland biopsy focus score >= 1.0/ 4 mm*mm; definition: multiple lymphocytic foci with >50 lymphocytes adjacent to mucinous acini, evaluated in 4 mm*mm of glandular tissue | pathology |
The diagnosis is made if either:[2]
- Four of six criteria required, must include either autoantibodies or histology.
- Three of the four objective (non-history) criteria are met.
Notes:
- ANA[3] and RF[4] were criteria in the past; they are no longer considered important in the diagnosis.
Microscopic
Features (salivary gland):[3]
- Viable salivary gland (or lacrimal gland) acini with significant lymphocytic infiltration - key feature.
- +/-Fibrosis.
DDx:
Note:
- Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient.
Images:
Focus score
- This is a count of significant lymphocytic foci.
Grading - historical
In the past lesions were graded with the Chisholm-Mason classification.[5]
It is based on assessing 4 mm2 area of salivary gland tissue and depends on the abundance and aggregation of lymphocytes as follows:[7]
| 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.
Sign out
LOWER LIP, BIOPSY: - SQUAMOUS MUCOSA WITH PARAKERATOSIS. - SALIVARY GLAND WITH RARE LYMPHOCYTES, NO LYMPHOEPITHELIAL LESIONS APPARENT. - NO FIBROSIS. COMMENT: The inflammation corresponds to Chisholm-Mason classification grade 0-1. Perivascular lymphocytes are seen focally. Clinical and serologic correlation is required.
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 Vitali, C.; Bombardieri, S.; Jonsson, R.; Moutsopoulos, HM.; Alexander, EL.; Carsons, SE.; Daniels, TE.; Fox, PC. et al. (Jun 2002). "Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group.". Ann Rheum Dis 61 (6): 554-8. PMC 1754137. PMID 12006334. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1754137/.
- ↑ 3.0 3.1 "Information from your family doctor. Sjögren syndrome.". Am Fam Physician 79 (6): 472. Mar 2009. PMID 19323360.
- ↑ 4.0 4.1 4.2 Vivino, FB.; Gala, I.; Hermann, GA. (May 2002). "Change in final diagnosis on second evaluation of labial minor salivary gland biopsies.". J Rheumatol 29 (5): 938-44. PMID 12022353.
- ↑ 5.0 5.1 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.
- ↑ URL: http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa. Accessed on: 24 July 2012.
- ↑ 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.