Sjögren's syndrome classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Farima Kahe M.D. [2]

American-European Consensus Group (AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to clinical findings.

• In 2002, American-European Consensus Group (AECG) established the Sjögren’s syndrome criteria based on the clinical observations, which include:^{[1][2][3][4][5]}
  • 1. Ocular symptoms: a positive result to at least one of the following questions:
    • 1.1. Have you had daily, persistent, troublesome dry eyes for more than 3 months?
    • 1.2. Do you have a recurrent sensation of sand or gravel in the eyes?
    • 1.3. Do you use tear substitutes more than 3 times a day?
  • 2. Oral symptoms: a positive result to at least one of the following questions:
    • 2.1. Have you had a daily feeling of dry mouth for more than 3 months?
    • 2.2. Have you had recurrently or persistently swollen salivary glands as an adult?
    • 2.3. Do you frequently drink liquids to aid in swallowing dry food?
  • 3. Ocular signs: positive result for at least one of the following two tests:
    • 3.1. Schirmer’s test, performed without anaesthesia (≤5 mm in 5 minutes)
    • 3.2. Rose bengal score or other ocular dye score (≥4 according to van Bijsterveld’s scoring system)
  • 4. Histopathology: number of lymphocytic foci score ≥1 focus/4 mm2 of glandular tissue
  • 5. Salivary gland involvement: positive result for at least one of the following diagnostic tests:
    • 5.1. Unstimulated whole salivary flow (≤1.5 ml in 15 min)
    • 5.2. Parotid sialography showing the presence of diffuse sialectasis (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts
    • 5.3. Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer
  • 6. Autoantibodies: presence in the serum of the following autoantibodies:
    • 6.1. Antibodies to Ro (SSA) or La (SSB) antigens, or both

• Exclusion criteria: if any of the following disorders is present, patients with the classification criteria are excluded.
  • Prior head and/or neck irradiation
  • Infection with hepatitis C virus (HCV)
  • Acquired immunodeficiency syndrome (AIDS)
  • Lymphoma
  • Sarcoidosis
  • Graft-versus-host disease
  • Recent use of medications with anticholinergic properties

• Sjögren’s syndrome may be classified as either primary or secondary:
  • Primary Sjögren’s syndrome
    • Positive salivary gland biopsy or anti-Ro/SSA and/or anti-La/SSB autoantibodies and have a total of four out of the six above items
    • Three out of the four objective items (ocular signs, biopsy, salivary gland involvement, or autoantibodies)
  • Secondary Sjögren’s syndrome
    • If a connective tissue disease is present, and if both of the following criteria are met:
      • Symptoms of ocular or oral dryness
      • Any two of the following three objective items:
        • Ocular signs
        • Positive salivary gland biopsy
        • Abnormal tests of salivary gland function

• American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2012 for at least two of the three following findings:^[6]
  • Positive serum anti-Ro/SSA and/or anti-La/SSB antibody testing OR a positive rheumatoid factor and an antinuclear antibody (ANA) titer ≥1:320
  • Ocular staining score ≥3 (assuming the individual is not concurrently using daily eye drops)
  • Presence of focal lymphocytic sialadenitis with a focus score ≥1 focus/4 mm2 in labial salivary gland biopsy samples