Criteria of Vitali et al for Sjogren's Syndrome (Preliminary European Criteria)

Criteria:

(1) ocular symptoms

(2) oral symptoms

(3) ocular signs

(4) histopathologic features

(5) salivary gland involvement

(6) presence of autoantibodies

Exclusions:

(1) pre-existing malignant lymphoma

(2) acquired immune deficiency syndrome (AIDS)

(3) sarcoidosis

(4) graft-vs-host disease (GVHD)

Ocular symptoms consist of a positive response to >= 1 of the following:

(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?

Oral symptoms consist of a positive response to >=1 of the following:

(1) Have you had a daily feeling of dry mouth for more than 3 months?

(2) Have you had recurrent or persistently swollen salivary glands as an adult?

(3) Do you frequently drink liquids to aid swallowing dry foods?

Ocular signs consist of 1 or both of the following:

(1) Schirmer-I test (<= 5 mm in 5 minutes)

(2) Rose bengal score >= 4 (van Bijsterveld scoring system)

Histopathology consists of focus score >= 1 in a minor salivary gland biopsy (with a focus defined as an aggregate of >= 50 mononuclear cells and the focus score the number of foci in a 4 square mm area of glandular tissue)

Salivary gland involvement with >= 1 of the following:

(1) enlarged salivary glands on scintigraphy

(2) parotid sialography

(3) unstimulated salivary flow <= 1.5 mL in 15 minutes

Autoantibodies with evidence of >= 1 of the following:

(1) anti-Ro/SS-A (for primary Sjogren's syndrome)

(2) anti-La/SS-B  (for primary Sjogren's syndrome)

(3) ANA (for secondary Sjogren's syndrome)

(4) rheumatoid factor (for secondary Sjogren's syndrome)

Criteria for primary Sjogren's Syndrome:

(1) >= 4 of the 6 criteria, with anti-Ro or anti-La

(2) no excluding conditions

Criteria for secondary Sjogren's Syndrome:

(1) not primary Sjogren's syndrome

(2) criterion 1 or 2 present

(3) number of criteria 3, 4, and 5 present >= 2

NOTE: The excluded conditions may or may not be viewed as causes of secondary Sjogren's syndrome (some might restrict the term secondary to patients with an autoimmune disease such as SLE or rheumatoid arthritis). This would determine how excluding conditions could be included into the definition of secondary Sjogren's syndrome.