Difference between revisions of "Sjögren syndrome"
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===Diagnostic criteria=== | ===Diagnostic criteria=== | ||
Four of six criteria required, must include either autoantibodies or histology:<ref>{{Cite journal | last1 = | Four of six criteria required, must include either autoantibodies or histology:<ref>{{Cite journal | last1 = Vitali | first1 = C. | last2 = Bombardieri | first2 = S. | last3 = Jonsson | first3 = R. | last4 = Moutsopoulos | first4 = HM. | last5 = Alexander | first5 = EL. | last6 = Carsons | first6 = SE. | last7 = Daniels | first7 = TE. | last8 = Fox | first8 = PC. | last9 = Fox | first9 = RI. | title = Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. | journal = Ann Rheum Dis | volume = 61 | issue = 6 | pages = 554-8 | month = Jun | year = 2002 | doi = | PMID = 12006334 | PMC =1754137 }} | ||
</ref> | |||
{| class="wikitable sortable" | {| class="wikitable sortable" | ||
! Criteria | ! Criteria | ||
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|- | |- | ||
| Oral symptoms | | Oral symptoms | ||
| any: (1) dry mouth > 3 months, (2) require fluids for swallowing, (3) swollen salivary glands | | any: (1) dry mouth > 3 months, (2) require fluids for swallowing, (3) swollen salivary glands (adults) | ||
| history | | history | ||
|- | |- | ||
| Oral signs | | Oral signs | ||
| any: (1) low salivary flow, (2) salivary scintigraphy positive (3) sialography positive | | any: (1) low salivary flow test positive, (2) salivary scintigraphy positive (3) (parotid) sialography positive | ||
| clinical test | | clinical test | ||
|- | |- | ||
| Ocular symptoms | | Ocular symptoms | ||
| any: (1) dry eye > 3 months, (2) need artifical tears >3x/day, (3) | | any: (1) dry eye > 3 months, (2) need artifical tears >3x/day, (3) sand or gravel in the eyes sensation | ||
| history | | history | ||
|- | |- | ||
| Ocular signs | | Ocular signs | ||
| any: (1) Schirmer's test positive, (2) | | any: (1) Schirmer's test positive, (2) ocular dye test positive | ||
| clinical test | | clinical test | ||
|- | |- | ||
| Autoantibodies | | Autoantibodies | ||
| | | anti-SSA/Ro and/or anti-SSB/La | ||
| serology | | serology | ||
|- | |- | ||
| Line 45: | Line 46: | ||
| pathology | | pathology | ||
|} | |} | ||
==Microscopic== | ==Microscopic== | ||
Revision as of 14:13, 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).
Blood work:[2]
- ANA +ve.
- Anti-SSA (Ro) +ve.
- Anti-SSB (La) +ve.
Diagnostic criteria
Four of six criteria required, must include either autoantibodies or histology:[3]
| 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/mm*mm | pathology |
Microscopic
Features (salivary gland):[2]
- Lymphocytic infiltration - key feature.
- "Benign lymphoepithelial lesion"[4] - intraepithelial lymphocytes.
- Viable salivary gland or lacrimal gland acini.
- +/-Plasma cells.
- +/-Fibrosis.
DDx:
Note:
- Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient.
- Perivascular lymphocytes not important.
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:[6]
| 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 "Information from your family doctor. Sjögren syndrome.". Am Fam Physician 79 (6): 472. Mar 2009. PMID 19323360.
- ↑ 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/.
- ↑ 4.0 4.1 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.