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Childhood-onset of primary Sjögren's syndrome: phenotypic characterization at diagnosis of 158 children

Ramos-Casals, M; Acar-Denizli, N; Vissink, A; Brito-Zerón, P; Li, X; Carubbi, F; Priori, R; ... Sjogren Big Data Consortium; + view all (2021) Childhood-onset of primary Sjögren's syndrome: phenotypic characterization at diagnosis of 158 children. Rheumatology 10.1093/rheumatology/keab032. (In press). Green open access

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Abstract

OBJECTIVES: To characterize the phenotypic presentation at diagnosis of childhood-onset primary Sjögren syndrome (SjS). METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SjS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 according to the fulfilment of the 2002/2016 classification criteria. RESULTS: Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary ultrasound study, 140/155 (90%) positive antinuclear antibody, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive rheumatoid factor. The systemic ESSDAI domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SjS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and central nervous system) in comparison with patients with adult-onset disease. CONCLUSIONS: Childhood-onset primary SjS involves around 1% of patients with primary SjS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role on modulating the phenotypic expression of the disease.

Type: Article
Title: Childhood-onset of primary Sjögren's syndrome: phenotypic characterization at diagnosis of 158 children
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/rheumatology/keab032
Publisher version: https://doi.org/10.1093/rheumatology/keab032
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Sjogren's Syndrome, Epidemiology, Autoimmune Diseases, Paediatrics, Childhood
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10122462
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