What is the American College of Rheumatology/European European League Against Rheumatism (ACR/EULAR) classification criteria used in the diagnosis of Sjögren syndrome?

Updated: Mar 05, 2021
  • Author: Sriya K Ranatunga, MD, MPH; Chief Editor: Herbert S Diamond, MD  more...
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Answer

According to the ACR/EULAR classification criteria, individuals are classified as having primary Sjögren syndrome if they have a total score of 4 or higher, derived from the sum of the weights assigned to the following [2] :

  • Focal lymphocytic sialadenitis and focus score of ≥1 foci/4 mm² in labial salivary gland biopsy samples  –  weight/score 3
  • Anti-SSA/Ro positive  – weight/score 3
  • Ocular Staining Score ≥5 (or van Bijsterveld score ≥4) in at least one eye  –  weight/score 1
  • Schirmer’s test ≤5 mm/5 min in at least one eye – weight/score 1
  • Unstimulated whole saliva flow rate ≤0.1 mL/min – weight/score 1

For inclusion, patients must have at least one symptom of ocular or oral dryness, defined as a positive response to at least one of the following questions:

  • Have you had daily, persistent, troublesome dry eyes for more than 3 months?
  • Do you have a recurrent sensation of sand or gravel in the eyes?
  • Do you use tear substitutes more than three times a day?
  • Have you had a daily feeling or dry mouth for more than 3 months?
  • Do you frequently drink liquids to aid in swallowing dry food?

Exclusion criteria include any of the following:

  • History of head-and-neck radiation treatment
  • Active hepatitis C infection (with confirmation by polymerase chain reaction [PCR]) testing
  • AIDS
  • Sarcoidosis
  • Amyloidosis
  • Graft versus host disease
  • IgG4-related disease

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