Difference between revisions of "Sjögren syndrome"
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'''Sjögren syndrome''', also '''Sjögren disease''', is | '''Sjögren syndrome''', also '''Sjögren disease''', is an uncommon disease that [[rheumatology|rheumatologists]] see. 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> | 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> | ||
Revision as of 15:15, 11 April 2013
Sjögren syndrome, also Sjögren disease, is an uncommon disease that rheumatologists see. 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 adjacent to a significant lymphocytic infiltrate - key feature.
- "Significant" lymphocytic infiltrate: cluster of >= 50 lymphocytes - important.
- Lymphocytes that are perivascular count.[4]
- Plasma cells should not exceed 10% of the inflammatory infiltrate.[4]
- May have "benign lymphoepithelial lesions"[5] - intraepithelial lymphocytes.
- "Significant" lymphocytic infiltrate: cluster of >= 50 lymphocytes - important.
- +/-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.