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'''Sjögren syndrome''', also '''Sjögren disease''', is | '''Sjögren syndrome''', also '''Sjögren disease''', is an uncommon disease that [[rheumatology|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''.<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== | ||
Line 8: | Line 12: | ||
*Dry mouth (xerostomia). | *Dry mouth (xerostomia). | ||
*Dry eyes (xerophthalmia). | *Dry eyes (xerophthalmia). | ||
===Diagnostic criteria=== | ===Diagnostic criteria=== | ||
European criteria of 2002:<ref name=pmid12006334>{{Cite journal | last1 = Vitali | first1 = C. | last2 = Bombardieri | first2 = S. | last3 = Jonsson | first3 = R. | last4 = Moutsopoulos | first4 = HM. | last5 = Alexander | first5 = EL. | last6 = Carsons | first6 = SE. | last7 = Daniels | first7 = TE. | last8 = Fox | first8 = PC. | last9 = Fox | first9 = RI. | title = Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. | journal = Ann Rheum Dis | volume = 61 | issue = 6 | pages = 554-8 | month = Jun | year = 2002 | doi = | PMID = 12006334 | PMC =1754137 }} | |||
</ref> | </ref> | ||
{| class="wikitable sortable" | {| class="wikitable sortable" | ||
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|- | |- | ||
| Histology | | Histology | ||
| labial minor salivary gland biopsy focus score > 1.0/mm*mm | | 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 | | pathology | ||
|} | |} | ||
The diagnosis is made if either:<ref name=pmid12006334/> | |||
#Four of six criteria required, must include either autoantibodies or histology. | |||
#Three of the four objective (non-history) criteria are met. | |||
Notes: | |||
*ANA<ref name=pmid19323360>{{Cite journal | title = Information from your family doctor. Sjögren syndrome. | journal = Am Fam Physician | volume = 79 | issue = 6 | pages = 472 | month = Mar | year = 2009 | doi = | PMID = 19323360 | URL = http://www.aafp.org/afp/2009/0315/p465.html }}</ref> and RF<ref name=pmid12022353>{{Cite journal | last1 = Vivino | first1 = FB. | last2 = Gala | first2 = I. | last3 = Hermann | first3 = GA. | title = Change in final diagnosis on second evaluation of labial minor salivary gland biopsies. | journal = J Rheumatol | volume = 29 | issue = 5 | pages = 938-44 | month = May | year = 2002 | doi = | PMID = 12022353 }}</ref> were criteria in the past; they are no longer considered important in the diagnosis. | |||
==Microscopic== | ==Microscopic== | ||
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": 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> | |||
* | ***[[Plasma cell]]s should not exceed 10% of the inflammatory infiltrate.<ref name=pmid12022353/> | ||
***May have "benign lymphoepithelial lesions"<ref name=pmid15956090/> - intraepithelial lymphocytes. | |||
*+/-Fibrosis. | *+/-Fibrosis. | ||
DDx: | DDx: | ||
*[[MALT lymphoma]]. | *[[MALT lymphoma]]. | ||
*[[Chronic sialadenitis]] - mixed inflammatory infiltrate. | |||
Note: | Note: | ||
*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> | ||
===Grading=== | ===Focus score=== | ||
Features:<ref name=pmid12022353/> | |||
*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.<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==== | |||
In the past lesions were graded with the ''Chisholm-Mason classification''.<ref name=pmid15956090>{{Cite journal | last1 = Ramos-Casals | first1 = M. | last2 = Font | first2 = J. | title = Primary Sjögren's syndrome: current and emergent aetiopathogenic concepts. | journal = Rheumatology (Oxford) | volume = 44 | issue = 11 | pages = 1354-67 | month = Nov | year = 2005 | doi = 10.1093/rheumatology/keh714 | PMID = 15956090 | url = http://rheumatology.oxfordjournals.org/content/44/11/1354.long }}</ref><ref>{{Cite journal | last1 = Chisholm | first1 = DM. | last2 = Mason | first2 = DK. | title = Labial salivary gland biopsy in Sjögren's disease. | journal = J Clin Pathol | volume = 21 | issue = 5 | pages = 656-60 | month = Sep | year = 1968 | doi = | PMID = 5697370 | PMC = 473887 | url = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC473887/?tool=pubmed }}</ref> The ''Chisholm-Mason classification'' is based on assessing 4 mm<sup>2</sup> area of salivary gland tissue and depends on the abundance and aggregation of lymphocytes. | |||
It is compares to Daniels-Whitcher grading as follows: | |||
{| class="wikitable sortable" | {| class="wikitable sortable" | ||
! | ! Lymphocytes | ||
! | ! Daniels-Whitcher <br>grade | ||
! Chisholm-Mason <br>grade | |||
|- | |- | ||
| slight infiltrate | | slight infiltrate | ||
| mild | |||
| 1 | |||
|- | |- | ||
| moderate infiltrate or less than one focus † | |||
| intermediate | |||
| 2 | | 2 | ||
|- | |- | ||
| one focus † | |||
| severe | |||
| 3 | | 3 | ||
|- | |- | ||
| more than one focus † | |||
| severe | |||
| 4 | | 4 | ||
|} | |} | ||
† Focus = aggregrate of 50 lymphocytes. | † Focus = an aggregrate of 50 lymphocytes or more. | ||
==Sign out== | ==Sign out== | ||
===Suggestive=== | |||
<pre> | <pre> | ||
LOWER LIP, BIOPSY: | LOWER LIP, BIOPSY: | ||
- | - MINOR SALIVARY GLAND WITH FOCAL LYMPHOCYTIC SIALADENITIS, SEE COMMENT. | ||
- SQUAMOUS MUCOSA WITH PARAKERATOSIS, MILD. | |||
- | |||
COMMENT: | COMMENT: | ||
The | The histologic findings are compatible with those seen in Sjoegren's disease | ||
(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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