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.
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 | history |
| Oral signs | any: (1) low salivary flow, (2) salivary scintigraphy positive (3) sialography positive | clinical test |
| Ocular symptoms | any: (1) dry eye > 3 months, (2) need artifical tears >3x/day, (3) foreign body sensation | history |
| Ocular signs | any: (1) Schirmer's test positive, (2) vital dye staining, (3) fluorescein tear breakup abn. | clinical test |
| Autoantibodies | any: (1) anti-SSA/Ro or anti-SSB/La, (2) ANA or RF (with other causes excluded) | 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.
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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.
- ↑ 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.
- ↑ 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.