American-European Consensus Sjögren’s Classification Criteria

The classification criteria for Sjögren’s syndrome (SS) currently used by clinicians and researchers around the world is the American-European Consensus Classification Criteria. Because many different criteria previously were used both within the U.S. and in other countries, the Sjögren’s Syndrome Foundation and members of the European Study Group on Classification Criteria brought international leaders in Sjögren’s together to develop consensus on one set of guidelines.1

Keep in mind that classification criteria is the strictest criteria available to prove a definitive diagnosis of Sjögren’s for research purposes. Physicians usually diagnose SS for clinical purposes on a more individual, medically intuitive and broader basis.

I. Ocular Symptoms (at least one)

  • Dry eyes >3 months?
  • Foreign body sensation in the eyes?
  • Use of artificial tears >3x per day?

II. Oral Symptoms (at least one)

  • Dry mouth >3 months?
  • Recurrent or persistently swollen salivary glands?
  • Need liquids to swallow dry foods?

III. Ocular Signs (at least one)

  • Schirmer's test, (without anesthesia) ≤5 mm/5 minutes
  • Positive vital dye staining (van Bijsterveld ≥4)

IV. Histopathology Lip biopsy showing focal lymphocytic sialoadenitis
     (focus score ≥1 per 4 mm2)2

 

V. Oral Signs (at least one)

  • Unstimulated whole salivary flow (≤1.5 mL in 15 minutes)
  • Abnormal parotid sialography3
  • Abnormal salivary scintigraphy4

VI. Autoantibodies (at least one)

  • Anti-SSA (Ro) or Anti-SSB (La)

For a primary Sjögren’s diagnosis:
a. Any 4 of the 6 criteria, must include either item IV (Histopathology) or VI (Autoantibodies)
b. Any 3 of the 4 objective criteria (III, IV, V, VI)

For a secondary Sjögren’s diagnosis:
In patients with another well-defined major connective tissue disease, the presence of one symptom (I or II) plus 2 of the 3 objective criteria (III, IV and V) is indicative of secondary SS.

Exclusion Criteria

  • Past head and neck radiation treatment
  • Hepatitis C infection
  • Acquired immunodeficiency syndrome (AIDS)
  • Pre-existing lymphoma
  • Sarcoidosis
  • Graft versus host disease
  • Current use of anticholinergic drugs

REFERENCES

  1. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH, European Study Group on Classification Criteria for Sjögren’s Syndrome. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002 Jun;61(6):554-8.
  2. Daniels TE, Whitcher JP. Association of patterns of labial salivary gland inflammation with keratoconjunctivitis sicca. Analysis of 618 patients with suspected Sjögren’s syndrome. Arthritis Rheum. 1994;37:869-877.
  3. Rubin H, Holt M. Secretory sialography in diseases of the major salivary glands. AJR Am J Roentgenol. 1957;77:575-598.
  4. Shall GL, Anderson LG, Wolf RO, Herdt JR, Tarpley TM Jr, Cummings NA, et al. Xerostomia in Sjögren’s syndrome: evaluation by sequential scintigraphy. JAMA. 1971;216:2109-2116.

 

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