Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Primary Sjögren’s syndrome (pSS) is a systemic auto-immune disease affecting the exocrine glands leading to sicca complaints. For the diagnosis of pSS, a positive salivary gland biopsy is an important criterion. A salivary gland biopsy can either be obtained from the minor (labial) or major (parotid) salivary gland and is considered positive if focus score (FS) is ≥1. In this study, the parotid gland biopsy (PGB) and labial gland biopsy (LGB) were compared for their diagnostic value in a prospective diagnostic cohort.
Methods: The study population consisted of 94 consecutive patients referred to the Sjögren center of expertise at the University Medical Center Groningen. All patients presented with sicca complaints and had a complete diagnostic workup for suspicion of pSS according to the American European Consensus Group (AECG) criteria. As part of the routine diagnostic workup a PGB was taken. In addition, patients consented for a simultaneous LGB. Biopsies were assessed for FS and presence of lymphoepithelial lesions (LELs).
Results: Of the included 94 patients (mean age (yr) 49.9 SD 13.9), 39 patients were classified as pSS according to the AECG criteria. In the patients classified as pSS, 18 patients (46%) had both salivary gland biopsies positive. In 14 patients (36%) only the LGB was positive, while in 3 patients (8%) only the PGB was positive and 4 patients (10%) both biopsies were negative. Using the AECG criteria, including salivary gland histology, the PGB had a higher specificity (95% vs 88%), but lower sensitivity (54% vs 82%) than the LGB. Patients classified as pSS, according to the AECG criteria, with both salivary gland biopsies positive, were all anti-SSA positive. Of the 14 patients classified as pSS with solely a positive LGB, 9 patients (64%) would not have been classified as pSS according to the AECG criteria if the biopsy had been negative, since these patients all lacked SSA or SSB autoantibodies. In the small group of three patients classified as pSS with a positive PGB and negative LGB, two patients were anti-SSA positive and the remaining anti-SSA negative patient (33%) would not have been classified as pSS if the biopsy was negative. The 4 classified pSS patients with both salivary gland biopsies negative were all anti-SSA positive. Thus, a positive LGB positively influenced the classification as pSS stronger than a positive PGB. Strikingly, although all 14 patients with solely a positive LGB showed a FS≥1, none of these LGBs harbored LELs. In comparison, if both salivary gland biopsies were positive, LELs were found in 79% of the LGBs. Patients with a positive LGB, but negative PGB were significantly older than patients with a positive PGB (57.0yr versus 47.2yr, t-test p=0.021).
Conclusion: This study suggests that anti-SSA negative elderly patients, with a positive labial gland biopsy based upon FS≥1, are at risk of being falsely diagnosed as pSS, since these labial gland biopsies all lack LELs. We therefore propose that LELs are taken into account in elderly patients besides FS≥1. A parotid gland biopsy is more specific and might be recommended especially in elderly patients without SSA antibodies, suspected for pSS.
To cite this abstract in AMA style:Haacke EA, van Nimwegen JF, van Ginkel MS, Arends S, Spijkervet FKL, Verstappen GM, Sillevis Smitt-Kamminga N, Vissink A, Kroese FGM, van der Vegt B, Bootsma H. Elderly Patients Risk a False Positive Diagnosis of Primary Sjogren’s Syndrome If a Positive Labial Gland Biopsy Is Solely Based on Focus Score [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/elderly-patients-risk-a-false-positive-diagnosis-of-primary-sjogrens-syndrome-if-a-positive-labial-gland-biopsy-is-solely-based-on-focus-score/. Accessed September 28, 2020.
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