Difference between revisions of "Sjögren syndrome"

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Sjögren syndrome (view source)

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'''Sjögren syndrome''', also '''Sjögren disease''', is a disease that keeps [[rheumatology|rheumatologists]] busy.  Sjögren is also spelled '''Sjoegren''' and '''Sjogren'''.   
'''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 9: Line 13:
*Dry eyes (xerophthalmia).
*Dry eyes (xerophthalmia).


Blood work:<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>
===Diagnostic criteria===
*ANA +ve.
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 }}
*Anti-SSA (Ro) +ve.
</ref>
*Anti-SSB (La) +ve.
{| class="wikitable sortable"
! 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:<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/>
*Lymphocytic infiltration - '''key feature'''.
*"Significant lymphocytic infiltrate" adjacent to viable [[salivary gland]] (or [[lacrimal gland]]) acini - '''key feature'''.
**"Benign lymphoepithelial lesion"<ref name=pmid15956090/> - intraepithelial lymphocytes.
**"Significant lymphocytic infiltrate": cluster of >= 50 lymphocytes - '''important'''.
*Viable [[salivary gland]] or [[lacrimal gland]] acini.
***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.
***[[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.
*Perivascular lymphocytes ''not'' important.


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)].


===Grading===
===Focus score===
Lesions can be 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> It is based on assessing 4 mm<sup>2</sup> area of salivary gland tissue and depends on the abundance and aggregation of lymphocytes as follows:<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>
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"  
!Grade
! Lymphocytes
!Lymphocytes
! Daniels-Whitcher <br>grade
! Chisholm-Mason <br>grade
|-
|-
| 0
| absent
|-
| 1
| slight infiltrate
| slight infiltrate
| mild
| 1
|-
|-
| moderate infiltrate or less than one focus †
| intermediate
| 2
| 2
| moderate infiltrate or less than one focus †
|-
|-
| one focus †
| severe
| 3
| 3
| one focus †
|-
|-
| more than one focus †
| severe
| 4
| 4
| more than one focus †
|}
|}
† 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:
- SQUAMOUS MUCOSA WITH PARAKERATOSIS.
- MINOR SALIVARY GLAND WITH FOCAL LYMPHOCYTIC SIALADENITIS, SEE COMMENT.
- SALIVARY GLAND WITH A MINIMAL PERIVASCULAR LYMPHOCYTIC INFILTRATE, NO LYMPHOEPITHELIAL LESIONS APPARENT.
- SQUAMOUS MUCOSA WITH PARAKERATOSIS, MILD.
- NO FIBROSIS.


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


Michael
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