Sjögren syndrome: Difference between revisions

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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" lymphocytic infiltrate": cluster of >= 50 lymphocytes - '''important'''.
**"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>
*[http://commons.wikimedia.org/wiki/File:Sjogren_syndrome_%281%29.jpg SS - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Sjogren_syndrome_%282%29.jpg SS - high mag. (WC)].


===Focus score===
===Focus score===
Features:<ref name=pmid12022353/>
Features:<ref name=pmid12022353/>
*This is nothing more than a count of significant lymphocytic foci.
*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.
**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):
# Count the significant foci (n).
# Estimate the sample area in mm<sup>2</sup> (a).
 
:<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:
- SQUAMOUS MUCOSA WITH PARAKERATOSIS.
- MINOR SALIVARY GLAND WITH FOCAL LYMPHOCYTIC SIALADENITIS, SEE COMMENT.
- SALIVARY GLAND WITH RARE LYMPHOCYTES, NO LYMPHOEPITHELIAL LESIONS APPARENT.
- SQUAMOUS MUCOSA WITH PARAKERATOSIS, MILD.
- NO FIBROSIS.


COMMENT:
COMMENT:
The inflammation corresponds to Chisholm-Mason classification grade 0-1.
The histologic findings are compatible with those seen in Sjoegren's disease
Perivascular lymphocytes are seen focally. Clinical and serologic correlation  
(focus score >= 1).
is required.
 
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.
</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>


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