Sjögren syndrome

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.

General

Clinical - classically:

• Women in 50s.
• Dry mouth (xerostomia).
• Dry eyes (xerophthalmia).

Blood work:

• ANA +ve.
• Anti-SSA (Ro) +ve.
• Anti-SSB (La) +ve.

Microscopic

Features (salivary gland):

• Lymphocytic infiltration - key feature.
  • "Benign lymphoepithelial lesion" - intraepithelial lymphocytes.
• Viable salivary gland or lacrimal gland acini.
• +/-Plasma cells.
• +/-Fibrosis.

DDx:

• MALT lymphoma.

Note:

• Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient.
• Perivascular lymphocytes not important.

Images:

• Sjögren syndrome (medscape.com).
• SS - low mag. (WC).
• SS - high mag. (WC).

Grading

Lesions can be graded with the Chisholm-Mason classification. It is based on assessing 4 mm² area of salivary gland tissue and depends on the abundance and aggregation of lymphocytes as follows:

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 PERIVASCULAR LYMPHOCYTIC INFILTRATE, NO LYMPHOEPITHELIAL LESIONS APPARENT.
- NO FIBROSIS.

COMMENT:
Clinical and serologic correlation is required. The inflammation corresponds 
to Chisholm-Mason classification grade 0-1.

See also

• Systemic lupus erythematosus.
• Scleroderma.
• Blood work.