Sjögren's syndrome classification: Difference between revisions

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==Overview==
==Overview==
 
American-European Consensus Group (AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings.


==Classification==
==Classification==
*American-European Consensus Group(AECG) established the Sjögren's syndrome criteria were based the clinical observations as following:
*In 2002, American-European Consensus Group (AECG) established the Sjögren's syndrome criteria based on the clinical observations, which include:<ref name="pmid12006334">{{cite journal |vauthors=Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH |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 |date=June 2002 |pmid=12006334 |pmc=1754137 |doi= |url=}}</ref><ref name="pmid12480687">{{cite journal |vauthors=Vitali C |title=Classification criteria for Sjögren's syndrome |journal=Ann. Rheum. Dis. |volume=62 |issue=1 |pages=94–5; author reply 95 |date=January 2003 |pmid=12480687 |pmc=1754291 |doi= |url=}}</ref><ref name="pmid18984413">{{cite journal |vauthors=Theander E, Jacobsson LT |title=Relationship of Sjögren's syndrome to other connective tissue and autoimmune disorders |journal=Rheum. Dis. Clin. North Am. |volume=34 |issue=4 |pages=935–47, viii–ix |date=November 2008 |pmid=18984413 |doi=10.1016/j.rdc.2008.08.009 |url=}}</ref><ref name="pmid7979575">{{cite journal |vauthors=Vitali C, Moutsopoulos HM, Bombardieri S |title=The European Community Study Group on diagnostic criteria for Sjögren's syndrome. Sensitivity and specificity of tests for ocular and oral involvement in Sjögren's syndrome |journal=Ann. Rheum. Dis. |volume=53 |issue=10 |pages=637–47 |date=October 1994 |pmid=7979575 |pmc=1005429 |doi= |url=}}</ref><ref name="pmid22563590">{{cite journal |vauthors=Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan J, Daniels T |title=American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort |journal=Arthritis Care Res (Hoboken) |volume=64 |issue=4 |pages=475–87 |date=April 2012 |pmid=22563590 |pmc=3349440 |doi= |url=}}</ref>
**'''1.Ocular symptoms:''' a positive result to at least one of the following questions:
**'''1. Ocular symptoms:''' a positive result to at least one of the following questions:
***1.1. Have you had daily, persistent, troublesome dry eyes for more than 3 months?
***1.1. Have you had daily, persistent, troublesome [[dry eyes]] for more than 3 months?
***1.2. Do you have a recurrent sensation of sand or gravel in the eyes?
***1.2. Do you have a recurrent [[sensation]] of sand or gravel in the [[Eye|eyes]]?
***1.3. Do you use tear substitutes more than 3 times a day?
***1.3. Do you use [[Tears|tear]] substitutes more than 3 times a day?
**'''2.Oral symptoms:''' a positive result to at least one of the following questions:
**'''2. Oral symptoms:''' a positive result to at least one of the following questions:
***2.1. Have you had a daily feeling of dry mouth for more than 3 months?
***2.1. Have you had a daily feeling of [[dry mouth]] for more than 3 months?
***2.2. Have you had recurrently or persistently swollen salivary glands as an adult?
***2.2. Have you had recurrently or persistently swollen [[salivary glands]] as an adult?
***2.3. Do you frequently drink liquids to aid in swallowing dry food?
***2.3. Do you frequently drink liquids to aid in [[swallowing]] dry food?
**'''3.Ocular signs:''' positive result for at least one of the following two tests:
**'''3. Ocular signs:''' positive result for at least one of the following two tests:
***3.1. Schirmer's test, performed without anaesthesia (≤5 mm in 5 minutes)
***3.1. [[Schirmer's test]], performed without [[anaesthesia]] (≤5 mm in 5 minutes)
***3.2. Rose bengal score or other ocular dye score (≥4 according to van Bijsterveld's scoring system)
***3.2. [[Rose bengal]] score or other [[ocular]] dye score (≥4 according to van Bijsterveld's scoring system)
**'''4.Histopathology:''' number of lymphocytic foci per 4 square mm of glandular tissue
**'''4. Histopathology:''' number of [[lymphocytic]] foci score ≥1 focus/4 mm2 of [[glandular tissue]]
**'''5.Salivary gland involvement:''' positive result for at least one of the following diagnostic tests:
**'''5. Salivary gland involvement:''' positive result for at least one of the following diagnostic tests:
***5.1. Unstimulated whole salivary flow (≤1.5 ml in 15 min)
***5.1. Unstimulated whole salivary flow (≤1.5 ml in 15 min)
***5.2. Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts
***5.2. [[Parotid]] [[sialography]] showing the presence of diffuse sialectasis (punctate, cavitary or destructive pattern), without evidence of [[obstruction]] in the major ducts
***5.3. Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer
***5.3. Salivary [[scintigraphy]] showing delayed uptake, reduced concentration and/or delayed [[excretion]] of tracer
**'''6.Autoantibodies:''' presence in the serum of the following autoantibodies:
**'''6. Autoantibodies:''' presence in the serum of the following [[autoantibodies]]:
***6.1.Antibodies to Ro(SSA) or La(SSB) antigens, or both
***6.1. Antibodies to Ro (SSA) or La (SSB) antigens, or both


*'''Exclusion criteria''': if any of the following disorders is present, patients with the classification criteria are excluded.
*'''Exclusion criteria''': if any of the following disorders is present, patients with the classification criteria are excluded.
**Prior head and/or neck irradiation
**Prior head and/or neck [[irradiation]]
**Infection with hepatitis C virus (HCV)
**[[Infection]] with [[hepatitis C virus]] ([[Hepatitis C|HCV]])
**Acquired immunodeficiency syndrome (AIDS)
**[[Acquired immunodeficiency syndrome]] ([[HIV AIDS|AIDS]])
**Lymphoma
**[[Lymphoma]]
**Sarcoidosis
**[[Sarcoidosis]]
**Graft-versus-host disease
**[[Graft-versus-host disease]]
**Recent use of medications with anticholinergic properties
**Recent use of [[Medication|medications]] with [[anticholinergic]] properties


*Sjögren's syndrome may be classified as either primary or secondary:
*Sjögren's syndrome may be classified as either primary or secondary:
**'''Primary Sjögren's syndrome'''
**'''Primary Sjögren's syndrome'''
***Positive salivary gland biopsy or anti-Ro/SSA and/or anti-La/SSB autoantibodies and satisfies a total of four of the six above items  
***Positive [[salivary gland]] [[biopsy]] or anti-Ro/SSA and/or anti-La/SSB [[autoantibodies]] and have a total of four out of the six above items  
***Three of the four objective items (ocular signs, biopsy, salivary gland involvement, or autoantibodies)
***Three out of the four objective items ([[ocular]] signs, [[biopsy]], [[salivary gland]] involvement, or [[autoantibodies]])
**'''Secondary Sjögren's syndrome'''
**'''Secondary Sjögren's syndrome'''
***If a connective tissue disease is present and if both of the following criteria are met:
***If a [[connective tissue]] disease is present, and if both of the following criteria are met:
****Symptoms of ocular or oral dryness
****Symptoms of [[ocular]] or [[oral]] dryness
****Any two of the following three objective items are present:
****Any two of the following three objective items:
*****Ocular signs
*****[[Ocular]] signs
*****Positive salivary gland biopsy
*****Positive [[salivary gland]] [[biopsy]]
*****Abnormal tests of salivary gland function
*****Abnormal tests of [[salivary gland]] function
 
*American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2012 for at least two of the three following findings:<ref name="pmid225635902">{{cite journal |vauthors=Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan J, Daniels T |title=American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort |journal=Arthritis Care Res (Hoboken) |volume=64 |issue=4 |pages=475–87 |date=April 2012 |pmid=22563590 |pmc=3349440 |doi= |url=}}</ref>
**Positive serum anti-Ro/SSA and/or anti-La/SSB [[antibody]] testing '''OR''' a positive [[rheumatoid factor]] and an [[antinuclear antibody]] ([[Antinuclear antibodies|ANA]]) titer ≥1:320
**[[Ocular]] staining score ≥3 (assuming the individual is not concurrently using daily eye drops)
**Presence of focal [[lymphocytic]] [[sialadenitis]] with a focus score ≥1 focus/4 mm2 in labial [[salivary gland]] [[biopsy]] samples
 
==References==
==References==
{{reflist|2}}
{{reflist|2}}


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Farima Kahe M.D. [2]

Overview

American-European Consensus Group (AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to clinical findings.

Classification

  • In 2002, American-European Consensus Group (AECG) established the Sjögren's syndrome criteria based on the clinical observations, which include:[1][2][3][4][5]
    • 1. Ocular symptoms: a positive result to at least one of the following questions:
      • 1.1. Have you had daily, persistent, troublesome dry eyes for more than 3 months?
      • 1.2. Do you have a recurrent sensation of sand or gravel in the eyes?
      • 1.3. Do you use tear substitutes more than 3 times a day?
    • 2. Oral symptoms: a positive result to at least one of the following questions:
      • 2.1. Have you had a daily feeling of dry mouth for more than 3 months?
      • 2.2. Have you had recurrently or persistently swollen salivary glands as an adult?
      • 2.3. Do you frequently drink liquids to aid in swallowing dry food?
    • 3. Ocular signs: positive result for at least one of the following two tests:
    • 4. Histopathology: number of lymphocytic foci score ≥1 focus/4 mm2 of glandular tissue
    • 5. Salivary gland involvement: positive result for at least one of the following diagnostic tests:
      • 5.1. Unstimulated whole salivary flow (≤1.5 ml in 15 min)
      • 5.2. Parotid sialography showing the presence of diffuse sialectasis (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts
      • 5.3. Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer
    • 6. Autoantibodies: presence in the serum of the following autoantibodies:
      • 6.1. Antibodies to Ro (SSA) or La (SSB) antigens, or both
  • American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2012 for at least two of the three following findings:[6]

References

  1. ↑ Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH (June 2002). "Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group". Ann. Rheum. Dis. 61 (6): 554–8. PMC 1754137. PMID 12006334.
  2. ↑ Vitali C (January 2003). "Classification criteria for Sjögren's syndrome". Ann. Rheum. Dis. 62 (1): 94–5, author reply 95. PMC 1754291. PMID 12480687.
  3. ↑ Theander E, Jacobsson LT (November 2008). "Relationship of Sjögren's syndrome to other connective tissue and autoimmune disorders". Rheum. Dis. Clin. North Am. 34 (4): 935–47, viii–ix. doi:10.1016/j.rdc.2008.08.009. PMID 18984413.
  4. ↑ Vitali C, Moutsopoulos HM, Bombardieri S (October 1994). "The European Community Study Group on diagnostic criteria for Sjögren's syndrome. Sensitivity and specificity of tests for ocular and oral involvement in Sjögren's syndrome". Ann. Rheum. Dis. 53 (10): 637–47. PMC 1005429. PMID 7979575.
  5. ↑ Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan J, Daniels T (April 2012). "American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort". Arthritis Care Res (Hoboken). 64 (4): 475–87. PMC 3349440. PMID 22563590.
  6. ↑ Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan J, Daniels T (April 2012). "American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort". Arthritis Care Res (Hoboken). 64 (4): 475–87. PMC 3349440. PMID 22563590.
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