Revised International Classification Criteria for Sjögren’s Syndrome (SS)

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 3 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 minutes)

  2. Rose bengal score or other ocular dye score (>/=4 according to van Bijsterveld’s scoring system)

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 a number of lymphocytic foci (which are adjacent to normal-appearing mucous acini and contain more than 50 lymphocytes) per 4 mm2 of glandular tissue

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 minutes)

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

Revised Rules for Classification

For primary 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 (that is, 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

For secondary SS

In patients with a potentially associated disease (for instance, another well defined connective tissue disease), the presence of item I or item II plus any 2 from among items III, IV, and V may be considered as indicative of secondary SS

Exclusion criteria:

  • Past head and neck radiation treatment

  • Hepatitis C infection

  • Acquired immunodeficiency disease (AIDS)

  • Pre-existing lymphoma

  • Sarcoidosis

  • Graft versus host disease

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

 

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 Sjogren’s Syndrome. Classification criteria for Sjogren’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. [Medline]

Created: Dic 08, 2005

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