Sjogren’s Syndrome

Sjogren’s Syndrome Criteria

The presence of any four of the following six criteria items provides sensitivity 97.4%, specificity 89.4%.

(1) Ocular symptoms: a positive response to at least one of the following questions:

• Have you had daily, persistent, troublesome dry eyes for more than 3 months?
• Do you have a recurrent sensation of sand or gravel in the eyes?
• Do you use tear substitutes more than 3 times a day?

(2) Oral symptoms: a positive response to at least one of the following questions:

• Have you had a daily feeling of dry mouth for more than 3 months?
• Have you had recurrently or persistently swollen salivary glands as an adult?
• Do you frequently drink liquids to aid in swallowing dry food?

(3) Ocular signs: objective evidence of ocular involvement defined as a positive result for at least one of the following two tests:

• Schirmer’s I test, performed without anesthesia (<5 mm in 5 minutes)
• Rose bengal score or another ocular dye score (>4 according to van Bijsterveld’s scoring system)

(4) Histopathology

• In minor salivary glands (obtained through normal-appearing mucosa) focal lymphocytic sialadenitis, 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.

(5) Salivary gland involvement: objective evidence of salivary gland involvement defined by a positive result for at least one of the following diagnostic tests:

• Unstimulated whole salivary flow (<1.5 ml in 15 minutes)
• Parotid sialography showing the presence of diffuse sialectasis (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts
• Salivary scintigraphy showing delayed uptake reduced concentration and/or delayed excretion of tracer 20

(6) Autoantibodies: presence in the serum of the following autoantibodies:

• Antibodies to Ro(SSA) or La(SSB) antigens, or both

PRIMARY SJOGREN’S

In patients without any potentially associated disease, primary SS may be defined as follows:

• 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
• The presence of any 3 of the 4 objective criteria items (that is, items III, IV, V, VI)

SECONDARY SJOGREN’S

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)