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Juvenile Sjögren syndrome: Clinical characteristics with focus on salivary gland ultrasonography

Arthritis Care & Research Dec 14, 2019

Hammenfors DS, Valim V, Bica BERG, et al. - Researchers sought to report on symptoms and clinical findings of juvenile Sjögren's syndrome (SS). Further, they investigated the clinical application of major salivary gland ultrasonography (SGUS) in patients with juvenile SS. In this cross-sectional multicenter study of 67 patients with disease onset until age 18 years, the female:male ratio was approximately 6.4:1. The first symptom was reported at the mean age of 10.2 years and the diagnosis was made at the mean age of 12.1 years. Forty-two of 67 patients (63%) and 53 of 66 patients (80%) had ocular and oral symptoms, respectively. Forty-two of 67 patients (63%) fulfilled the American-European Consensus Group or American College of Rheumatology/European League Against Rheumatism classification criteria for primary SS. Forty-one of 67 patients (61%) exhibited pathologic SGUS findings; primary SS criteria were fulfilled by 26 of 41 SGUS+ patients (63%). Thirty-seven of 58 patients exhibited salivary gland enlargements/parotitis which was nonsignificantly associated with SGUS+ status. The mean levels of saliva were 5.6 ml/15 minutes and 3.3 ml/15 minutes in SGUS– patients vs the SGUS+ patients. A total of 36 of 41 SGUS+ patients (88%) were anti-Ro/La+ relative to 14 of 26 SGUS– patients (54%). In addition, rheumatoid factor positivity was reported in 24 of 39 SGUS+ patients (62%) vs 5 of 25 SGUS– patients (20%). These findings suggest that a large spectrum of clinical symptoms and findings characterize juvenile SS. Pathologic SGUS findings were evident in correlation to several glandular and extraglandular parameters such as hyposalivation, swollen salivary glands, and autoantibodies.
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