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Juvenile Sjögren's Syndrome: Clinical Characteristics With Focus on Salivary Gland Ultrasonography
Author(s) -
Hammenfors Daniel S.,
Valim Valéria,
Bica Blanca E. R. G.,
Pasoto Sandra G.,
Lilleby Vibke,
NietoGonzález Juan Carlos,
Silva Clovis A.,
Mossel Esther,
Pereira Rosa M. R.,
Coelho Aline,
Bootsma Hendrika,
Thatayatikom Akaluck,
Brun Johan G.,
Jonsson Malin V.
Publication year - 2020
Publication title -
arthritis care and research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.032
H-Index - 163
eISSN - 2151-4658
pISSN - 2151-464X
DOI - 10.1002/acr.23839
Subject(s) - medicine , rheumatism , rheumatology , ultrasonography , saliva , gastroenterology , surgery
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.