Criteria

American-European Consensus Group classification criteria[69][83]

The American-European Consensus Group (AECG) classification criteria (American-European criteria) have been used since 2002 as a new gold standard to ensure specific diagnosis.

Requires 3 of 4 objective criteria:

  • Objective ocular signs: Schirmer's test, rose bengal testing or lissamine green and fluorescein

  • Involvement of salivary gland by functional testing: salivary scintigraphy, parotid sialography

  • Anti-Ro ± Anti-La auto-antibodies

  • Histopathology on salivary gland biopsy.

Or 4 of 6 criteria, of which at least 1 of anti-Ro, anti-La, and/or histopathology must be present:

  • Objective ocular signs (i.e., Schirmer's test)

  • Involvement of salivary gland by functional testing (sialometry)

  • Auto-antibodies anti-Ro or anti-La

  • Histopathology on salivary gland biopsy

  • Oral symptoms

  • Ocular symptoms.

American College of Rheumatology classification criteria for Sjogren syndrome[83][84]​​

According to these research criteria, to be classified with Sjogren syndrome, at least 2 of the following criteria need to be met:

  • Positive anti-Ro60/SSA antibody and/or anti-La/SSB (or positive rheumatoid factor and antinuclear antibody titre >1:320)

  • Ocular staining score >3

  • Focal lymphocytic sialadenitis with a focus score >1 focus per 4 mm² in biopsy of labial salivary glands.

2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjogren’s syndrome[35]

Although the feasibility of the AECG criteria is superior to that of the American College of Rheumatology (ACR) criteria, their performance was similar among patients with systemic autoimmune diseases, and a subset of patients with Sjogren syndrome is still missed by both criteria sets.[83]​ Therefore, the ACR and the European League Against Rheumatism (EULAR) developed a data-driven single set of consensus classification criteria for primary Sjogren syndrome (pSS).[35]

Patients can be classified as having pSS if they meet the inclusion criteria*, and do not have any of the conditions listed as exclusion criteria**, and have a score ≥4 when the weights from the five criteria items below are summed up:

Weight/score = 3

  • Labial salivary gland with focal lymphocytic sialadenitis and focus score of ≥1 foci/4 mm²

  • Anti-Ro/SSA positive

Weight/score = 1

  • Ocular staining score ≥5 (or van Bijsterveld score ≥4) in at least one eye

  • Schirmer's test ≤5 mm/5 minutes in at least one eye

  • Unstimulated whole saliva flow rate ≤0.1 mL/minute

* Inclusion criteria

These criteria are applicable to any patient with 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 of dry mouth for more than 3 months?

  • Do you frequently drink liquids to aid in swallowing dry food?

Or in whom there is suspicion of Sjogren syndrome from the EULAR SS Disease Activity Index questionnaire (at least one domain with positive item).

**Exclusion criteria

  • History of head and neck radiation treatment

  • Active hepatitis C infection (with positive polymerase chain reaction [PCR])

  • Acquired immunodeficiency syndrome

  • Sarcoidosis

  • Amyloidosis

  • Graft-versus-host disease

  • IgG4-related disease

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