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 tissue18 |
|
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 ducts19 |
3. Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer20 |
|
VI. Autoantibodies: presence in the serum of the following autoantibodies: |
1. Antibodies to Ro(SSA) or La(SSB) antigens, or both |