Sjögren syndrome (SS), although commonly associated with xerostomia and xerophthalmia, can present with extraglandular manifestations that make definitive diagnosis difficult. The 2 leading diagnostic classification systems include a positive minor salivary gland biopsy (MSGB) result as a major inclusion criterion. The purpose of this study was to better define the role of the MSGB in establishing a diagnosis of SS in a cohort of referred patients.
This is a retrospective cohort study of patients referred to the Massachusetts General Hospital Department of Oral and Maxillofacial Surgery (Boston, MA) over a 5-year period to establish a diagnosis of SS. Inclusion criteria were complete information regarding presenting symptoms, reason for referral, and findings at MSGB. Incomplete records and referral for reasons other than presumptive SS resulted in exclusion. The MSGB and its role in definitive diagnosis based on accepted classification systems were evaluated for sensitivity, specificity, and positive and negative predictive values. Other variables of interest (demographics, pattern of referral, and symptomology) are described for the cohort.
Eighty-seven patients met the inclusion criteria. The mean age was 48.3 years (range, 19 to 74 yr) and 75 were women (86.2%). Fifteen MSGB results (17.2%) were positive based on the focus score histologic criteria. In 12 of 15 cases, the positive MSGB result allowed for a definitive diagnosis (80%). In 3 cases, other criteria allowed diagnosis without the contribution of the MSGB (sensitivity of MSGB, 80.0%; specificity, 87.5%; positive predictive value, 57.1%; negative predictive value, 95.5%).
The MSGB is an important contributor to a diagnosis of SS. When serology was negative, the MSGB often was the criterion that allowed the diagnosis to be established. It is the most frequently positive of the major criteria used by the 2 classification systems in current use.