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.

Microscopic

Features (salivary gland):[2]

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.

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LOWER LIP, BIOPSY:
- SQUAMOUS MUCOSA WITH PARAKERATOSIS.
- SALIVARY GLAND WITH A MINIMAL LYMPHOCYTIC INFILTRATE, SEE COMMENT.

COMMENT:
Clinical and serologic correlation is required. The inflammation corresponds 
to Chisholm-Mason classification grade 1.

See also

References

  1. 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. 2.0 2.1 "Information from your family doctor. Sjögren syndrome.". Am Fam Physician 79 (6): 472. Mar 2009. PMID 19323360.
  3. URL: http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa. Accessed on: 24 July 2012.
  4. 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.
  5. 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.