Criteria for the diagnosis of Sjogren's syndrome - both of the following:
(1) keratoconjunctivitis sicca
(2) xerostomia
If only 1 feature is present, then the patient may have incipient Sjogren's syndrome and should continue to be monitored.
Criteria for keratoconjunctivitis sicca (KCS) - 2 or 3 of the following:
(1) Schirmer test <= 10 mm in 5 minutes
(2) tear break up time <= 10 seconds
(3) Rose Bengal (van Bijsterveld) score >= 4 in range of 0 to 9
Criteria for xerostomia - 2 or 3 of the following:
(1) unstimulated saliva flow (sialometry) <= 1.5 mL in 15 minutes
(2) sialoadenitis in biopsy of a labial salivary gland (2 or more foci of >= 50 lymphoplasmohistiocytic cells in 4 square mm)
(3) abnormal salivary gland scintigraphy (diminished uptake, spontaneous secretion, OR secretion following citrous stimulation)
An objective test for keratoconjunctivitis sicca or xerostomia can be replaced by another provided the test is reproducible and has a high sensitivity and specificity.
If the patient has a preceding chronic inflammatory connective tissue disease, then the Sjogren's syndrome is termed secondary. This may include:
(1) rheumatoid arthritis (RA)
(2) systemic lupus erythematosus (SLE)
(3) progressive systemic sclerosis (scleroderma)
If this no evidence of another chronic inflammatory connective tissue disease, then the Sjogren's syndrome is termed primary.
