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)

Related Criteria

Revised ARA Criteria for the Classification of Rheumatoid Arthritis (RA)

Classification Criteria for the Diagnosis of Systemic Lupus Erythematosus (SLE)

Criteria for the Classification of Systemic Sclerosis (Scleroderma)

ACR Criteria for the Classification of Polyarteritis Nodosa (PAN)

ACR Criteria for the Classification of Churg-Strauss Syndrome (CSS)

ACR Criteria for the Classification of Giant-Cell Arteritis

ACR Criteria for the Classification of Hypersensitivity Vasculitis

1990 Criteria of American College of Rheumatology for the Classification of Takayasu Arteritis

Classification Criteria for Osteoarthritis

Diagnostic Criteria for Relapsing Polychondritis

Criteria for the Classification of Wegener's Granulomatosis (WG)

Diagnostic Criteria for Autoimmune Hepatitis (AIH)

Diagnostic Criteria for Kawasaki Disease

Names and definitions of vasculitis adopted by the Chapel Hill Consensus Conference

Diagnostic Criteria for Mixed Connective Tissue Disease (MCTD)

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More...

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]

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