Sjögren's syndrome classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Classification
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 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 square mm 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 min) 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