Table 1

Comparison of the Revised American–European Consensus Group (AECG) Classification criteria and the American College of Rheumatology (ACR) Classification criteria for Sjögren's Syndrome (SS)

AECG classification*ACR classification†
Inclusion criteriaInclusion criteria
I. Ocular symptoms: a positive response to at least one of the following questions:
  •  1. Have you had daily, persistent, troublesome dry eyes for more than 3 months?

  •  2. Do you have a recurrent sensation of sand or gravel in the eyes?

  •  3. Do you use tear substitutes more than three times a day?

II. Oral symptoms: a positive response to at least one of the following questions:
  •  1. Have you had a daily feeling of dry mouth for more than 3 months?

  •  2. Have you had recurrently or persistently swollen salivary glands as an adult?

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

III. Ocular signs—that is, objective evidence of ocular involvement defined as a positive result for at least one of the following two tests:
  •  1. Schirmer's I test, performed without anaesthesia (≤5 mm in 5 min)

  •  2. Rose Bengal score or other ocular dye score (≥4 according to van Bijsterveld's scoring system)

Keratoconjunctivitis sicca with ocular staining score ≥3 (assuming that individual is not currently using daily eye drops for glaucoma and has not had corneal surgery or cosmetic eyelid surgery in the last 5 years)
IV. Histopathology: in minor salivary glands (obtained through normal appearing mucosa) focal lymphocytic sialoadenitis, evaluated by an expert histopathologist, with a focus score ≥1, defined as number of lymphocytic foci (which are adjacent to normal-appearing mucous acini and contain more than 50 lymphocytes) per 4 mm2 of glandular tissueLabial salivary gland biopsy exhibiting focal lymphocytic sialadenitis with a focus score ≥1 focus/4 mm2
V. Salivary gland involvement: objective evidence of salivary gland involvement defined by a positive result for at least one of the following diagnostic tests:
  •  1. Unstimulated whole salivary flow (≤1.5 mL in 15 min)

  •  2. Parotid sialography‡ showing the presence of diffuse sialectasias (punctate, cavitary or destructive pattern), without evidence of obstruction in major ducts

  •  3. Salivary scintigraphy§ showing delayed uptake, reduced concentration and/or delayed excretion of tracer

VI. Autoantibodies: presence in the serum of the following autoantibodies:
  •  1. Antibodies to Ro (SSA) or La (SSB) antigens, or both

Positive serum anti-SSA/Ro and/or anti-SSB/La or (positive rheumatoid factor and ANA titre ≥1:320)
Classification rulesClassification rules
For primary SS:For SS:
In patients without any potentially associated disease, primary SS may be defined as follows:
  1. The presence of any 4 of the 6 items is indicative of primary SS, as long as either item IV (histopathology) or VI (serology) is positive

  2. The presence of any 3 of the 4 objective criteria items (ie, items III, IV, V, VI)

  3. The classification tree procedure represents a valid alternative method for classification, although it should be more properly used in clinical-epidemiological survey

The classification of SS, which applies to individuals with signs/symptoms that may be suggestive of SS, will be met in patients who have at least 2 of the 3 objective features previously described
For Secondary SS:
In patients with a potentially associated disease (for instance, another well-defined connective tissue disease), the presence of item 1 or item II plus any 2 from among items III, IV and V may be considered as indicative of secondary SSEliminated the distinction between primary and secondary forms of SS
Exclusion criteriaExclusion criteria
  •  1. Past head and neck radiation treatment

  •  2. Hepatitis C infection

  •  3. AIDS

  •  4. Pre-existing lymphoma

  •  5. Sarcoidosis

  •  6. Graft versus host disease

  •  7. Use of anticholinergic drugs (since a time shorter than 4-fold the half-life of the drug)

Prior diagnosis of any of the following conditions would exclude participation in SS studies or therapeutic trials because of overlapping clinical features or interference with criteria tests:
  •  1. History of head and neck radiation treatment

  •  2. Hepatitis C infection

  •  3. AIDS

  •  4. Sarcoidosis

  •  5. Amyloidosis

  •  6. Graft versus host disease

  •  7. IgG4-related disease

  • *Revised AECG classification criteria.19

  • †ACR criteria.21

  • ‡Sialography: this test was not performed in the current study.

  • §Scintigraphy: this test was not performed in the current study.

  • ANA, antinuclear antibodies.