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2016 ACR/EULAR Classification Criteria for Primary Sjogren's Syndrome
Weighted classification criteria for primary Sjogren's syndrome in patients with sicca symptoms.
2016 ACR/EULAR Classification Criteria for Primary Sjogren's Syndrome
Exclusion criteria present (prior head/neck radiation, active hepatitis C infection, AIDS, sarcoidosis, amyloidosis, graft-versus-host disease, or IgG4-related disease)?
The 2016 criteria only apply once an alternative cause of sicca symptoms has been excluded.
Labial salivary gland biopsy with focal lymphocytic sialadenitis, focus score >=1 focus/4 mm^2
Anti-SSA/Ro antibody positive
Ocular Staining Score >=5 (or van Bijsterveld score >=4) in at least one eye
Schirmer's test <=5 mm/5 min in at least one eye
Unstimulated whole saliva flow rate <=0.1 mL/min
Result0 points
Score <4: does not fulfil the classification criteria for primary Sjogren's syndrome.
- Weighted criteria score
- 0
When to use
- Classifying patients who have at least one symptom of ocular or oral dryness (or in whom Sjogren's syndrome is suspected on the EULAR Sjogren's Syndrome Disease Activity Index) for research/trial enrollment.
- Not intended to replace clinical judgement for individual diagnosis.
Formula
Sum of weighted items (only applied when inclusion criteria are met and exclusion criteria are absent): labial salivary gland biopsy with focal lymphocytic sialadenitis (focus score >=1/4mm^2) = 3; anti-SSA/Ro positive = 3; Ocular Staining Score >=5 (or van Bijsterveld >=4) = 1; Schirmer's test <=5 mm/5 min = 1; unstimulated whole salivary flow <=0.1 mL/min = 1. Total score >=4 classifies as primary Sjogren's syndrome.
Pearls and pitfalls
- Developed and validated using three international patient cohorts (SICCA, Sjogren International Collaborative Clinical Alliance, and others), with sensitivity 96% and specificity 95% (both >=95%) against expert-adjudicated diagnoses in the validation cohort.
- A positive anti-SSA/Ro or a positive labial salivary gland biopsy each independently contribute the most weight to the score.