Entry Criterion - Required Before Scoring
Apply these criteria to patients with at least one of: (1) ocular dryness (eyes feel dry daily for more than 3 months, recurring sensation of sand/gravel in eyes, or use of tear substitutes more than 3 times/day), (2) oral dryness (daily feeling of dry mouth for more than 3 months, recurrently swollen salivary glands, or needing liquids to swallow dry foods), OR (3) positive anti-SSA/Ro antibody, OR (4) findings suspicious on minor salivary gland biopsy. Also: exclude IgG4-RD, active HCV, HIV/AIDS, sarcoidosis, amyloidosis, GVHD, and prior head/neck radiation.
1
Lip Biopsy - Minor Salivary Gland
Minor salivary gland biopsy showing focal lymphocytic sialadenitis with a focus score of 1 or more foci per 4 mm squared. This single item scores 3 points - the highest-weighted item in the criteria.
No biopsy performed, or biopsy not showing focal lymphocytic sialadenitis with focus score >=1/4mm2
0 pts
Focal lymphocytic sialadenitis with focus score >=1 foci/4 mm squared on lip biopsy
This is the single highest-weighted criterion (3 points). A focus is defined as an aggregate of 50 or more lymphocytes in periductal or perivascular areas of the gland parenchyma.
3 pts
Anti-SSA/Ro positivity by any validated assay. This also scores 3 points - equal to the biopsy criterion. Together, biopsy and anti-SSA together score 6 of the 4-point threshold, meaning either alone is sufficient for classification when combined with one additional item.
Anti-SSA/Ro negative or not tested
0 pts
Anti-SSA/Ro antibody positive
Tested by ELISA, immunodiffusion, or equivalent validated assay. Anti-Ro60 and anti-Ro52 both qualify. A positive result contributes 3 points - the maximum for any single item.
3 pts
Objective assessment of ocular surface damage. Van Bijsterveld score >=4 or ocular staining score (OSS) >=5 in at least one eye qualifies for 1 point.
Ocular staining score <5 (OSS) or van Bijsterveld <4 in both eyes, or not performed
0 pts
Ocular staining score >=5 (OSS) OR van Bijsterveld score >=4 in at least one eye
Staining with lissamine green and fluorescein. OSS grades corneal and conjunctival fluorescein staining (0-6 each area). Van Bijsterveld grades rose bengal staining of nasal conjunctiva, temporal conjunctiva, and cornea (0-9 total).
1 pt
Schirmer's test without anesthesia: strip wetting of 5 mm or less in 5 minutes in at least one eye. Scores 1 point when positive.
Schirmer's test >5 mm/5 min in both eyes, or not performed
0 pts
Schirmer's test <=5 mm/5 min in at least one eye (without anesthesia)
The patient wears the Schirmer strip for 5 minutes without topical anesthesia. Wetting of 5 mm or less is abnormal and scores 1 point. This tests lacrimal gland secretory function.
1 pt
5
Unstimulated Whole Salivary Flow Rate
Unstimulated whole saliva collected over 15 minutes. Flow rate of 0.1 mL/min or less qualifies. Alternatively, a positive scintigraphy or parotid sialography result also qualifies for this criterion.
Unstimulated salivary flow >0.1 mL/min, or not performed
0 pts
Unstimulated whole saliva flow rate <=0.1 mL/min
Collect saliva for 15 minutes with the patient seated, head tilted forward. Total volume divided by 15 gives mL/min. 0.1 mL/min or less is abnormal. This tests salivary gland secretory function.
1 pt
📊 Interpreting the Score
| Score | Classification | Notes |
|---|
| 0 to 3 | Does Not Meet Criteria | Insufficient evidence for primary Sjogren's disease classification. |
| 4 or more | Classifies as Primary Sjogren's | Sensitivity 96%, specificity 95% in the validation cohort of 1362 patients. |
Maximum score is 9 (lip biopsy 3 + anti-SSA 3 + ocular staining 1 + Schirmer 1 + salivary flow 1). A patient with positive anti-SSA (3 pts) and positive lip biopsy (3 pts) scores 6 - well above the threshold even without the functional tests.
💡 Pearls and Pitfalls
✓
Anti-SSA/Ro and lip biopsy are equally weighted at 3 points each. Either alone, combined with one minor criterion (ocular staining, Schirmer, or salivary flow), is sufficient for classification. Anti-SSA is more commonly available in clinical practice, making it the dominant classification driver in most cohorts.
✓
Seronegative Sjogren's still exists. Approximately 25-30% of primary Sjogren's patients are anti-SSA negative. These patients require a positive lip biopsy to reach the threshold without serology. Seronegative patients typically have milder systemic involvement but can still have significant sicca symptoms.
⚠
Strictly exclude IgG4-related disease. IgG4-RD can produce salivary gland swelling, sicca symptoms, and positive minor salivary gland biopsies that may mimic Sjogren's. IgG4-RD is an explicit exclusion criterion. When in doubt, stain the biopsy for IgG4-positive plasma cells and measure serum IgG4.
🔮 Evidence
The 2016 ACR/EULAR criteria were developed by Shiboski, Seror, Criswell, Mariette, and the Sjogren's Classification Criteria Project Consortium, and published in Arthritis and Rheumatology in 2017. The criteria replaced the 2002 American-European Consensus Group (AECG) criteria and the 2012 ACR criteria by unifying them and validating in a large international cohort of 1362 patients from 22 centers. Sensitivity 96% and specificity 95% represent substantial improvement over prior criteria.
View References▾
1
Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjogren's syndrome. Arthritis Rheumatol. 2017;69(1):35-45. Development and validation in 1362 patients.
2
Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61(6):554-558. Prior AECG criteria that the 2016 criteria replaced.
For classification purposes only. Score of 4 or more classifies primary Sjogren's disease after confirming the entry criterion and excluding competing diagnoses (IgG4-RD, HCV, HIV, sarcoidosis, amyloidosis, GVHD, prior head/neck radiation). Not a diagnostic tool.