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Arthritis Care Res (Hoboken). 2019 Jan 30. doi: 10.1002/acr.23839. [Epub ahead of print]

Juvenile Sjögren's syndrome: clinical characteristics with focus on salivary gland ultrasonography.

Author information

1
Department of Clinical Science - Section for Rheumatology, University of Bergen, Norway.
2
Department of Rheumatology, Haukeland University Hospital, University of Bergen, Norway.
3
Broegelmann Research Laboratory, Department of Clinical Science, University of Bergen, Norway.
4
Department of Rheumatology/Medicine, Federal University of Espírito Santo, Brazil.
5
Department of Rheumatology, Hospital Universitário Clementino Fraga Filho, Federal University of Rio de Janeiro, Brazil.
6
Sjögren's syndrome outpatient/Rheumatology Division/Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Brazil.
7
Department of Rheumatology, Oslo University Hospital, Norway.
8
Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
9
Department of Pediatric Rheumatology, Instituto da Crianca/ Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Brazil.
10
Department of Rheumatology and Clinical Immunology, University Medical Centre Groningen, University of Groningen, The Netherlands.
11
Rheumatology Division/Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Brazil.
12
Division of Allergy/Immunology/Rheumatology, Department of Pediatrics, University of Florida.
13
Department of Clinical Dentistry - Section for Oral and Maxillofacial Radiology, University of Bergen, Norway.

Abstract

BACKGROUND/OBJECTIVES:

Juvenile Sjögren's syndrome (jSS) is a rare, poorly defined and possibly underdiagnosed condition affecting children/adolescents. The aim of this study was to characterize symptoms and clinical findings of jSS, and to explore the clinical application of major salivary gland ultrasonography (SGUS) in patients with jSS.

METHODS:

A cross-sectional multicenter study recruited patients with disease onset until 18 years of age (n=67). Disease characteristics were recorded, unstimulated whole sialometry and SGUS examination of the parotid and submandibular salivary glands was performed.

RESULTS:

Female:male ratio was 58:9 (6.4:1). Mean age at first symptom was 10.2 years, 12.1 years at diagnosis. Ocular and oral symptoms were noted in 42/67 (63%) and 53/66 (80%) patients, respectively. AECG or ACR-EULAR classification criteria for primary SS (pSS) was fulfilled by 42/67 (63%) patients. Pathological SGUS findings (SGUS+) were observed in 41/67 (61%) patients; 26/41 (63%) SGUS+ fulfilled pSS criteria. Salivary gland enlargements/parotitis was noted in 37/58 patients and non-significantly associated with SGUS+ (p=0.066). Mean levels of saliva was 5.6 ml/15 minutes in SGUS- patients compared to 3.3 ml/15 minutes in the SGUS+ patients (p=0.049). 36/41 (88%) SGUS+ patients were anti-Ro/La+ compared to 14/26 (54%) SGUS- patients (p=0.001). 24/39 (62%) SGUS+ patients were RF+, whereas only 5/25 (20%) SGUS- patients were RF+ (p=0.001).

CONCLUSIONS:

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 pathological SGUS findings.

SIGNIFICANCE AND INNOVATION:

Interest for jSS is increasing and international collaborations are emerging. To date, this is the largest cohort world-wide characterizing juvenile Sjögren's syndrome and also the first large study investigating salivary gland ultrasonography in this patient group. This article is protected by copyright. All rights reserved.

PMID:
30697959
DOI:
10.1002/acr.23839

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