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.

Pathologists are likely to this condition as a labial salivary gland biopsy.

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

• 

  SS - low mag. (WC)

• 

  SS - high mag. (WC)

www:

• Sjögren syndrome (medscape.com).

Focus score

Features:

• This is nothing more than a count of significant lymphocytic foci per 4 mm*mm.
  • Significant: >= 50 lymphocytes, adjacent to (viable) salivary gland acini.
  • A focus score of one or more is considered significant.

Calculating the focus score (fs):

1. Count the significant foci (n).
2. Estimate the sample area in mm² (a).

$fs={n \over a}\times 4\ mm^{2}$

Grading

In the past lesions were graded with the Chisholm-Mason classification. The Chisholm-Mason classification is based on assessing 4 mm² area of salivary gland tissue and depends on the abundance and aggregation of lymphocytes.

It is compares to Daniels-Whitcher grading as follows:

Lymphocytes	Daniels-Whitcher grade	Chisholm-Mason grade
slight infiltrate	mild	1
moderate infiltrate or less than one focus †	intermediate	2
one focus †	severe	3
more than one focus †	severe	4

† Focus = an aggregrate of 50 lymphocytes or more.

Sign out

Suggestive

LOWER LIP, BIOPSY:
- MINOR SALIVARY GLAND WITH FOCAL LYMPHOCYTIC SIALADENITIS, SEE COMMENT.
- SQUAMOUS MUCOSA WITH PARAKERATOSIS, MILD.

COMMENT:
The histologic findings are compatible with those seen in Sjoegren's disease
(focus score >= 1).

SALIVARY GLAND - SUMMARY:
Glandular area: 8 mm*mm.
Interstitial fat: not apparent.
Plasma cells: not apparent.
Fibrosis: none apparent (0% of salivary gland area).
Ductular dilation: not apparent.
Gross foci: 4.
Focus score (foci/4 mm*mm): 2.

This result needs to be combined with the clinical and serologic criteria used
to diagnose Sjoegren's disease.

Not suggestive

Minor Salivary Gland, Biopsy:
- Benign minor salivary gland with one focus of chronic 
  lymphocyte predominant inflammation.
- Focus score (FS) much less than one; not suggestive of 
  Sjoegren's disease.

Comment:
Estimated area of salivary gland: 50 mm*mm. 
Number of foci (~50 lymphocytes): 1.
Plasma cells: not apparent.
Fibrosis: none/minimal.
Focus score (focus / area x 4 mm*mm) = << 1.

The findings do not exclude Sjoegren's disease. Clinical and serologic 
correlation is required.

See also

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