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

Abstract

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.

Figure 1

Representative major salivary gland ultrasonography images of submandibular glands, illustrating A, grade 0; B, grade 1; C, grade 2; and D, grade 3, with grades 0–1 corresponding to normal‐appearing morphology, and grades 2–3 corresponding to pathologic changes in the submandibular and parotid glands of patients with clinical symptoms of juvenile Sjögren's syndrome.

Figure 2

Total major salivary gland ultrasonography score (range 0–12) for all 4 glands in relation to the A, Total points of the American College of Rheumatology/European League Against Rheumatism classification criteria (range 0–9) (P = 0.016), B, Focus score, and C, Unstimulated whole saliva flow (range 0–21.36 ml/15 minute) (P = 0.015). The correlations were not significant after Benjamin‐Hochberg adjustment. We did not find a correlation with the focus score (range 0–8).