Classification criteria Sjögrens' syndrome 4.21/5 (14)

Last updated: 8 / 12 / 20
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ACR / EULAR Criteria 2016 (Shiboski CH, 2016)

Based on persons with dryness and / or similar Sjögren's suspected symptoms *

  • Focus score at biopsy at least = 1.0 foci / 4 square mm 3 points
  • Positive anti-SSA (Ro) antibody: 3 points
  • Color score / corneas at least 5 in at least one eye (or van Bijsterveld score at least 4): 1 point
  • Schirmer's test 5mm / 5 min or lower, at least one eye: 1 point
  • Unstimulated sialometry 0,1ml / min = 1,5ml / 15min: 1 point
  • At least 4 points = Sjögren's syndrome
    • In practice, either anti-SSA / Ro or positive tissue sample must be available

* At least one positive answer to the following 5 questions:

  1. Have you had daily, persistently troubled dry eyes for at least three months?
  2. Have you had a repetitive sense of sand or gravel in your eyes?
  3. Do you use artificial tear fluid at least three times a day?
  4. Have you had a daily feeling of dry mouth for at least three months?
  5. Do you often drink to swallow dry foods?

Classification criteria often used for diagnosis:

US / EU-crit. (reference: Vitali, C, 2002): 4 of 6 of which point 4. or point 6 are mandatory:

  1. At least one typical eye symptom (daily dryness)
  2. At least one typical mouth symptom (daily dryness)
  3. Schirmer's test, Rose-Bengal or dyeing positive at the ophthalmologist
  4. Lip biopsy (focus score ≥1, ie at least 50X lymphocytes / 4mm2)
  5. Sialometry positive
  6. Anti-SSA / Ro or anti-SSB / La present in blood samples

Former head / neck radiation therapy, Hepatitis C infection, HIV infection and use of special drugs (anticholinergic drugs for Parkinson's disease).

English version and reference here

Secondary Sjögren's syndrome

  1. There must be systemic connective tissue disease (SLE, Systemic sclerosis, Rheumatoid arthritis, MCTD, Myositis, Autoimmune liver disease, autoimmune thyroid disease). In addition, the following is required:
    1. Symptoms of dry eye or mouth dryness
    2. Two of the following:
      1. Eye dryness detected by medical examinations
      2. Typical changes in salivary gland biopsy
      3. Reduced saliva production during testing

Suggested criteria (ACR 2012, Shiboski SC et al Arthritis & Care Vol 64 April 2012 pp 475-487)

At least 2 of 3:

  1. anti-SSA / Ro and / or anti-SSB / La or rheumatoid factor (RF) or ANA ≥320 (blood sample)
  2. Lip biopsy with focus score ≥ 1 (tissue sample)
  3. Corneal changes (ophthalmologist) with color score ≥ 3 (assuming eye drops are not used daily and or corneal surgery last 5 years)

Exclusion: head / neck irradiation, hepatitis C, AIDS, sarcoidosis, amyloidosis, graft vs host, IgG4-related disease.

Criteria for Sjøgren`s Syndrome 1965-2016

  • Bloch KJ 1965
  • Daniels TE 1975
  • Skopouli FN 1986 (Greek criteria)
  • Fox RI 1986 (California criteria)
  • Manthorpe R 1986 (Copenhagen criteria)
  • Segerberg-Kottinen 1986
  • Vitali C 1993 (European criteria)
  • Vitali C 2002 (ACR / EU criteria)
  • Fujibayashi T 2004 (Japan Criteria)
  • Shiboski SC 2012 (ACR Criteria)
  • Shiboski SC 2016 (ACR / EULAR criteria)
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