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:
- Have you had daily, persistently troubled dry eyes for at least three months?
- Have you had a repetitive sense of sand or gravel in your eyes?
- Do you use artificial tear fluid at least three times a day?
- Have you had a daily feeling of dry mouth for at least three months?
- Do you often drink to swallow dry foods?
- Exclusion: Earlier sradiation therapy against head / neck, active Hepatitis C infection, AIDS infection, Sarcoidosis, Amyloidosis, Graft versus Host Disease, IgG4-related disease
- Use of special drugs, anticholinergic drugs, which causes dryness should be stopped well before measurement of tear and salivary gland function
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:
- At least one typical eye symptom (daily dryness)
- At least one typical mouth symptom (daily dryness)
- Schirmer's test, Rose-Bengal or dyeing positive at the ophthalmologist
- Lip biopsy (focus score ≥1, ie at least 50X lymphocytes / 4mm2)
- Sialometry positive
- 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).
Secondary Sjögren's syndrome
- 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:
- Symptoms of dry eye or mouth dryness
- Two of the following:
- Eye dryness detected by medical examinations
- Typical changes in salivary gland biopsy
- Reduced saliva production during testing
- Reference: Theander E, 2008
Suggested criteria (ACR 2012, Shiboski SC et al Arthritis & Care Vol 64 April 2012 pp 475-487)
At least 2 of 3:
- anti-SSA / Ro and / or anti-SSB / La or rheumatoid factor (RF) or ANA ≥320 (blood sample)
- Lip biopsy with focus score ≥ 1 (tissue sample)
- 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)