Sjögren syndrome

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.

General

Clinical - classically:

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

Diagnostic criteria

European criteria of 2002:

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:

1. Four of six criteria required, must include either autoantibodies or histology.
2. Three of the four objective (non-history) criteria are met.

Notes:

• ANA and RF were criteria in the past; they are no longer considered important in the diagnosis.

Microscopic

Features (salivary gland):

• "Significant lymphocytic infiltrate" adjacent to viable salivary gland (or lacrimal gland) acini - key feature.
  • "Significant" lymphocytic infiltrate": cluster of >= 50 lymphocytes - important.
    • Lymphocytes may be perivascular or periductular.
    • Plasma cells should not exceed 10% of the inflammatory infiltrate.
    • May have "benign lymphoepithelial lesions" - intraepithelial lymphocytes.
• +/-Fibrosis.

DDx:

• MALT lymphoma.
• Chronic sialadenitis - mixed inflammatory infiltrate.

Note:

• Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient.

Images:

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

Focus score

Features:

• This is nothing more than a count of significant lymphocytic foci.
  • Significant: >= 50 lymphocytes, adjacent to salivary gland acini.
  • One or more is considered significant.

Grading - historical

In the past lesions were 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:

Chisholm-Mason 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

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