Calculators & Tools

Sjögren Syndrome — ACR/EULAR 2016

Weighted classification criteria; a total of ≥ 4 classifies primary Sjögren syndrome.

1 What is the Sjögren Syndrome — ACR/EULAR 2016?

The Sjögren Syndrome — ACR/EULAR 2016 is a rheumatology scoring tool used in paediatric practice. Weighted classification criteria; a total of ≥ 4 classifies primary Sjögren syndrome. It scores 5 criteria and interprets the total across 2 bands, so the number always arrives with the severity it implies rather than on its own. In PediAid each criterion is shown as a labelled option with the weight it carries, so the total can be checked rather than taken on trust.

2 When to use it

  • Grading severity at the bedside in rheumatology
  • Recording an objective severity band in the case notes
  • Deciding when a total crosses the threshold for escalation
  • Re-scoring to track whether a child is improving or deteriorating
  • Checking the exact criteria of a score used infrequently

3 Formula & method

The score sums 5 criteria: Labial salivary gland biopsy with focal lymphocytic sialadenitis, focus score ≥ 1 foci/4 mm²; Anti-SSA/Ro antibody positive; Ocular staining score ≥ 5 (or van Bijsterveld ≥ 4) in at least one eye; Schirmer test ≤ 5 mm/5 min in at least one eye; Unstimulated whole saliva flow rate ≤ 0.1 mL/min. Totals are then banded: any score - Does not classify: total <4 - does not classify as primary Sjögren syndrome; 4 points or more - Classifies as Sjögren: total ≥4 - classifies as primary Sjögren syndrome (sensitivity 96%, specificity 95%). In children, recurrent parotitis is a more common presentation than sicca symptoms.
  • Applies to patients with at least one symptom of ocular or oral dryness, or suspicion from the ESSDAI. Exclusions include prior head/neck radiation, active hepatitis C, AIDS, sarcoidosis, amyloidosis, GVHD and IgG4-related disease.
  • Source: Shiboski CH et al. 2016 ACR/EULAR classification criteria for primary Sjögren's syndrome. Ann Rheum Dis 2017;76:9-16.

4 Frequently asked questions

How many criteria does the Sjögren Syndrome — ACR/EULAR 2016 use?

5. They are Labial salivary gland biopsy with focal lymphocytic sialadenitis, focus score ≥ 1 foci/4 mm²; Anti-SSA/Ro antibody positive; Ocular staining score ≥ 5 (or van Bijsterveld ≥ 4) in at least one eye; Schirmer test ≤ 5 mm/5 min in at least one eye; Unstimulated whole saliva flow rate ≤ 0.1 mL/min.

What do the bands of the Sjögren Syndrome — ACR/EULAR 2016 mean?

The total is interpreted as: any score - Does not classify: total <4 - does not classify as primary Sjögren syndrome; 4 points or more - Classifies as Sjögren: total ≥4 - classifies as primary Sjögren syndrome (sensitivity 96%, specificity 95%). In children, recurrent parotitis is a more common presentation than sicca symptoms.

Does the score replace clinical judgement?

No. It is a structured way to record and communicate severity. A clinician concerned about a child should act on that concern regardless of the total.

Where does this score come from?

Applies to patients with at least one symptom of ocular or oral dryness, or suspicion from the ESSDAI. Exclusions include prior head/neck radiation, active hepatitis C, AIDS, sarcoidosis, amyloidosis, GVHD and IgG4-related disease.

Does it work offline?

Yes. Score definitions are bundled with the PediAid app, so the score computes without a connection.

For qualified clinicians. This page and the PediAid app are a clinical aid only. Calculations and reference data must be verified against the patient's clinical context, the source guideline and your local protocols before any treatment decision is made.