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

Daniel S Hammenfors, Valéria Valim, Blanca E R G Bica, Sandra G Pasoto, Vibke Lilleby, Juan Carlos Nieto-González, Clovis A Silva, Esther Mossel, Rosa M R Pereira, Aline Coelho, Hendrika Bootsma, Akaluck Thatayatikom, Johan G Brun, Malin V Jonsson
Arthritis Care & Research 2019 January 30

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.

Full Text Links

Find Full Text Links for this Article


You are not logged in. Sign Up or Log In to join the discussion.

Trending Papers

Remove bar
Read by QxMD icon Read

Save your favorite articles in one place with a free QxMD account.


Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"