Sjögren's syndrome classification

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

Classification

• American-European Consensus Group(AECG) established the Sjögren's syndrome criteria were based the clinical observations as following:
  • 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 per 4 square mm 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 sialectasias (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 satisfies a total of four of the six above items
    • Three of the four objective items (ocular signs, biopsy, salivary gland involvement, or autoantibodies)
  • Secondary Sjögren's syndrome
    • Symptoms of ocular or oral dryness
    • Any two of the following three objective items are present:
      • Ocular signs
      • Positive salivary gland biopsy
      • Abnormal tests of salivary gland function