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Objective  Juvenile Sjögren's syndrome ( SS ) is a rare, poorly defined, and possibly underdiagnosed condition affecting children and adolescents. The aim of this study was to characterize symptoms and clinical findings of juvenile  SS  and to explore the clinical application of major salivary gland ultrasonography ( SGUS ) in patients with juvenile  SS .    Methods  A cross‐sectional multicenter study recruited patients with disease onset until age 18 years (n = 67). Disease characteristics were recorded, and unstimulated whole sialometry and  SGUS  examination of the parotid and submandibular salivary glands were performed.    Results  The female:male ratio was 58:9. The mean age at first symptom was 10.2 years and 12.1 years at diagnosis. Ocular and oral symptoms were noted in 42 of 67 patients (63%) and 53 of 66 patients (80%), respectively. The American‐European Consensus Group or American College of Rheumatology/European League Against Rheumatism classification criteria for primary  SS  were fulfilled by 42 of 67 patients (63%). Pathologic  SGUS  findings were observed in 41 of 67 patients (61%); 26 of 41  SGUS + patients (63%) fulfilled primary  SS  criteria. Salivary gland enlargements/parotitis were noted in 37 of 58 patients and were nonsignificantly associated with  SGUS + status ( P  = 0.066). The mean levels of saliva were 5.6 ml/15 minutes in  SGUS – patients compared to 3.3 ml/15 minutes in the  SGUS + patients ( P  = 0.049). A total of 36 of 41  SGUS + patients (88%) were anti‐Ro/La+ compared to 14 of 26  SGUS – patients (54%) ( P  = 0.001). In addition, 24 of 39  SGUS + patients (62%) were positive for rheumatoid factor ( RF ), whereas only 5 of 25  SGUS – patients (20%) were  RF + ( P  = 0.001).    Conclusion  Juvenile  SS  is characterized by a large spectrum of clinical symptoms and findings. Several glandular and extraglandular parameters such as hyposalivation, swollen salivary glands, and autoantibodies are associated with pathologic  SGUS  findings.

Juvenile Sjögren's Syndrome: Clinical Characteristics With Focus on Salivary Gland Ultrasonography