We evaluated the diagnostic accuracy of labial salivary gland specimens from a group of patients with symptoms or signs of dry mouth and/or dry eyes referred for assessment of possible Sjögren's syndrome (SS).
Fifty-eight individuals (52 women, 6 men; median age 54.5 yrs, range 19-90) had previously undergone one (n = 58) or 2 (n = 2) labial salivary gland biopsies, serologic studies, and objective tests for dry eyes and/or dry mouth to diagnose possible SS. Patients were referred to our institution for a second opinion regarding diagnosis and/or management of SS. All biopsy specimens underwent blinded review to measure aggregate glandular area, identify lymphocytic foci, and calculate focus scores that might verify the submitted diagnoses. Results were classified according to accepted histologic criteria: chronic sialadenitis, focal lymphocytic sialadenitis, indeterminate, insufficient tissue for diagnosis, and within normal limits. Institutional sources of submitted diagnoses included university hospitals (n = 26), university affiliates (n = 9), community hospitals (n = 18), commercial laboratories (n = 6), and a governmental agency (n = 1).
Upon reexamination, 32 of 60 accessions (53%) sustained a revision of the initial diagnosis. Application of the focus scoring system combined with clinical features to reveal 12 hitherto undocumented cases of SS and refuted the diagnosis of SS in 8 instances. The principal reason for inaccurate initial interpretation was failure to apply the focus scoring system in 58 of 60 instances. Median diagnostic delay for the 12 SS cases was 302 days (range 55-2821).
It is possible that widespread cross-institutional failure to apply the focus scoring system in the interpretation of labial salivary gland biopsies may delay the recognition and/or treatment of SS.