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Difference between revisions of "Sjögren syndrome"
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The syndrome may be part of another [[connective tissue disorder]], e.g. [[rheumatoid arthritis]], in which case it is called ''secondary Sjögren syndrome''.<ref name=pmid1703737>{{Cite journal | last1 = Celenligil | first1 = H. | last2 = Kansu | first2 = E. | last3 = Ruacan | first3 = S. | last4 = Eratalay | first4 = K. | last5 = Irkeç | first5 = M. | title = Characterization of peripheral blood and salivary gland lymphocytes in secondary Sjögren's syndrome. | journal = Ann Dent | volume = 49 | issue = 2 | pages = 18-22 | month = | year = 1990 | doi = | PMID = 1703737 }}</ref> | The syndrome may be part of another [[connective tissue disorder]], e.g. [[rheumatoid arthritis]], in which case it is called ''secondary Sjögren syndrome''.<ref name=pmid1703737>{{Cite journal | last1 = Celenligil | first1 = H. | last2 = Kansu | first2 = E. | last3 = Ruacan | first3 = S. | last4 = Eratalay | first4 = K. | last5 = Irkeç | first5 = M. | title = Characterization of peripheral blood and salivary gland lymphocytes in secondary Sjögren's syndrome. | journal = Ann Dent | volume = 49 | issue = 2 | pages = 18-22 | month = | year = 1990 | doi = | PMID = 1703737 }}</ref> | ||
Pathologists are likely to see this condition as a ''[[labial salivary gland]]'' biopsy. | |||
''Lip biopsy'' redirects here. | |||
==General== | ==General== | ||
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Features ([[salivary gland]]):<ref name=pmid19323360/> | Features ([[salivary gland]]):<ref name=pmid19323360/> | ||
*"Significant lymphocytic infiltrate" adjacent to viable [[salivary gland]] (or [[lacrimal gland]]) acini - '''key feature'''. | *"Significant lymphocytic infiltrate" adjacent to viable [[salivary gland]] (or [[lacrimal gland]]) acini - '''key feature'''. | ||
**"Significant | **"Significant lymphocytic infiltrate": cluster of >= 50 lymphocytes - '''important'''. | ||
***Lymphocytes may be perivascular or periductular.<ref name=pmid12022353/><ref name=pmid8003059>{{Cite journal | last1 = Daniels | first1 = TE. | last2 = Whitcher | first2 = JP. | title = Association of patterns of labial salivary gland inflammation with keratoconjunctivitis sicca. Analysis of 618 patients with suspected Sjögren's syndrome. | journal = Arthritis Rheum | volume = 37 | issue = 6 | pages = 869-77 | month = Jun | year = 1994 | doi = | PMID = 8003059 }}</ref> | ***Lymphocytes may be perivascular or periductular.<ref name=pmid12022353/><ref name=pmid8003059>{{Cite journal | last1 = Daniels | first1 = TE. | last2 = Whitcher | first2 = JP. | title = Association of patterns of labial salivary gland inflammation with keratoconjunctivitis sicca. Analysis of 618 patients with suspected Sjögren's syndrome. | journal = Arthritis Rheum | volume = 37 | issue = 6 | pages = 869-77 | month = Jun | year = 1994 | doi = | PMID = 8003059 }}</ref> | ||
***[[Plasma cell]]s should not exceed 10% of the inflammatory infiltrate.<ref name=pmid12022353/> | ***[[Plasma cell]]s should not exceed 10% of the inflammatory infiltrate.<ref name=pmid12022353/> | ||
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*Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient. | *Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient. | ||
Images: | ===Images=== | ||
<gallery> | |||
Image:Sjogren_syndrome_%281%29.jpg | SS - low mag. (WC) | |||
Image:Sjogren_syndrome_%282%29.jpg | SS - high mag. (WC) | |||
</gallery> | |||
www: | |||
*[http://img.medscape.com/pi/emed/ckb/rheumatology/329097-1339496-332125-1582482.jpg Sjögren syndrome (medscape.com)].<ref>URL: [http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa]. Accessed on: 24 July 2012.</ref> | *[http://img.medscape.com/pi/emed/ckb/rheumatology/329097-1339496-332125-1582482.jpg Sjögren syndrome (medscape.com)].<ref>URL: [http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa]. Accessed on: 24 July 2012.</ref> | ||
===Focus score=== | ===Focus score=== | ||
Features:<ref name=pmid12022353/> | Features:<ref name=pmid12022353/> | ||
*This is nothing more than a count of significant lymphocytic foci per 4 mm*mm. | *This is nothing more than a count of significant lymphocytic foci per 4 mm*mm. | ||
**Significant: >= 50 lymphocytes, adjacent to salivary gland acini. | **Significant: >= 50 lymphocytes, adjacent to (viable) [[salivary gland]] acini. | ||
**A ''focus score'' of one or more is considered significant.<ref name=pmid12006334/> | **A ''focus score'' of one or more is considered significant.<ref name=pmid12006334/> | ||
Calculating the focus score (fs): | Calculating the focus score (fs): | ||
# Count the significant foci (n). | # Count the significant foci (n). | ||
# Estimate | # Estimate the sample area in mm<sup>2</sup> (a). | ||
:<math>fs = { n \over a } \times 4 \ mm</math> | :<math>fs = { n \over a } \times 4 \ mm^2</math> | ||
====Grading==== | ====Grading==== | ||
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==Sign out== | ==Sign out== | ||
===Suggestive=== | |||
<pre> | <pre> | ||
LOWER LIP, BIOPSY: | LOWER LIP, BIOPSY: | ||
- MINOR SALIVARY GLAND WITH FOCAL LYMPHOCYTIC SIALADENITIS, SEE COMMENT. | - MINOR SALIVARY GLAND WITH FOCAL LYMPHOCYTIC SIALADENITIS, SEE COMMENT. | ||
- SQUAMOUS MUCOSA WITH PARAKERATOSIS, MILD. | - SQUAMOUS MUCOSA WITH PARAKERATOSIS, MILD. | ||
COMMENT: | COMMENT: | ||
Glandular area: | 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. | Interstitial fat: not apparent. | ||
Plasma cells: not apparent. | Plasma cells: not apparent. | ||
Fibrosis: none apparent (0% of salivary gland area). | Fibrosis: none apparent (0% of salivary gland area). | ||
Ductular dilation: not apparent. | Ductular dilation: not apparent. | ||
Gross foci: | Gross foci: 4. | ||
Focus score (foci/4 mm*mm): | Focus score (foci/4 mm*mm): 2. | ||
This result needs to be combined with the clinical and serologic criteria used | This result needs to be combined with the clinical and serologic criteria used | ||
to diagnose Sjoegren's disease. | to diagnose Sjoegren's disease. | ||
</pre> | |||
===Not suggestive=== | |||
<pre> | |||
Minor Salivary Gland, Biopsy: | |||
- Benign minor salivary gland with one focus of chronic lymphocyte | |||
predominant inflammation. | |||
- Focus score 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: few (<10%). | |||
Fibrosis: none/minimal. | |||
Focus score (number of foci / area x 4 mm*mm) = < 1. | |||
The findings do not exclude Sjoegren's disease. Clinical and serologic | |||
correlation is required. | |||
</pre> | </pre> | ||