Sjögren's syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Classification

Revised Classification Criteria for Sjogren's Syndrome

A. 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?

B. 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?

C. 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 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 )

D. 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.

E. 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

F. 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:

a. The presence of any 4 of the 6 items is indicative of primary SS, as long as either item 4 (Histopathology) or 6 (Serology) is positive.

b. The presence of any 3 of the 4 objective criteria items (that is, items 3, 4, 5, 6)

c. 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 1 or item 2 plus any two from among items 3, 4, and 5 may be considered as indicative of secondary SS.

Exclusive criteria

Past head and neck radiation therapy

Hepatitis C infection

Acquired immunodeficiency syndrome (AIDS)

Pre-existing lymphoma

Sarcoidosis

Graft-versus-host disease

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