Sjögren's syndrome classification
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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:
- 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:
- 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)
- 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
- Exclusion criteria: if any of the following disorders is present, patients with the classification criteria are excluded.
- Prior head and/or neck irradiation
- Infection with hepatitis C virus (HCV)
- Acquired immunodeficiency syndrome (AIDS)
- Lymphoma
- Sarcoidosis
- Graft-versus-host disease
- Recent use of medications with anticholinergic properties
- Sjögren's syndrome may be classified as either primary or secondary:
- Primary Sjögren's syndrome
- 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 out of the four objective items (ocular signs, biopsy, salivary gland involvement, or autoantibodies)
- Secondary Sjögren's syndrome
- If a connective tissue disease is present, and if both of the following criteria are met:
- Symptoms of ocular or oral dryness
- Any two of the following three objective items:
- Ocular signs
- Positive salivary gland biopsy
- Abnormal tests of salivary gland function
- If a connective tissue disease is present, and if both of the following criteria are met:
- Primary Sjögren's syndrome
- American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2012 for at least two of the three following findings:[6]
- Positive serum anti-Ro/SSA and/or anti-La/SSB antibody testing OR a positive rheumatoid factor and an antinuclear antibody (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
- ↑ 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.
- ↑ Vitali C (January 2003). "Classification criteria for Sjögren's syndrome". Ann. Rheum. Dis. 62 (1): 94–5, author reply 95. PMC 1754291. PMID 12480687.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.