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]

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

  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. 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. 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.
  5. URL: http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa. Accessed on: 24 July 2012.
  6. 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.