Journal article
Childhood-onset of primary Sjogren's syndrome: phenotypic characterization at diagnosis of 158 children
Rheumatology (Oxford, England), Vol.60(10), pp.4558-4567
10/02/2021
DOI: 10.1093/rheumatology/keab032
Abstract
Objectives To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. Methods The Big Data Sjogren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria. Results Among the 12 083 patients included in the Sjogren 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 US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjogren's syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS 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 CNS) in comparison with patients with adult-onset disease. Conclusions Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.
Details
- Title: Subtitle
- Childhood-onset of primary Sjogren's syndrome: phenotypic characterization at diagnosis of 158 children
- Creators
- Manuel Ramos-Casals - Universitat de BarcelonaNihan Acar-Denizli - Universitat Politècnica de CatalunyaArjan Vissink - University Medical Center GroningenPilar Brito-Zeron - Cellex (United States)Xiaomei Li - Anhui Provincial HospitalFrancesco Carubbi - University of L'AquilaRoberta Priori - Sapienza University of RomeNatasa Toplak - Ljubljana University Medical CentreChiara Baldini - University of PisaEnrique Faugier-Fuentes - Hospital Infantil de México Federico GómezAike A. Kruize - Utrecht UniversityThomas Mandl - Skåne University HospitalMinako Tomiita - National Hospital OrganizationSaviana Gandolfo - Ospedale Santa Maria della Misericordia di UdineKunio Hashimoto - Nagasaki UniversityGabriela Hernandez-Molina - Instituto Nacional de Ciencias Médicas y Nutrición Salvador ZubiránBenedikt Hofauer - Technical University of MunichSamara Mendieta-Zeron - Hospital Materno-InfantilAstrid Rasmussen - Oklahoma Medical Research FoundationPulukool Sandhya - Christian Medical College, VelloreDamien Sene - Hôpital LariboisièreVirginia Fernandes Moca Trevisani - Universidade Federal de São PauloDavid Isenberg - University College LondonErik Sundberg - Karolinska University HospitalSandra G. Pasoto - Hospital das Clínicas da Faculdade de Medicina da Universidade de São PauloAgata Sebastian - Wroclaw Medical UniversityYasunori Suzuki - Kanazawa University HospitalSoledad Retamozo - Instituto de Investigaciones en Ciencias de la SaludBei Xu - Anhui Provincial HospitalRoberto Giacomelli - University of L'AquilaAngelica Gattamelata - Sapienza University of RomeMasa Bizjak - Ljubljana University Medical CentreStefano Bombardieri - University of PisaRichard-Eduardo Loor-Chavez - Hospital Infantil de México Federico GómezAnneline Hinrichs - Utrecht UniversityPeter Olsson - Skåne University HospitalHendrika Bootsma - University Medical Center GroningenScott M. Lieberman - Univ Iowa, Carver Coll Med, Stead Family Dept Pediat, Iowa City, IA USASjogren Big Data Consortium
- Resource Type
- Journal article
- Publication Details
- Rheumatology (Oxford, England), Vol.60(10), pp.4558-4567
- DOI
- 10.1093/rheumatology/keab032
- ISSN
- 1462-0324
- eISSN
- 1462-0332
- Publisher
- Oxford Univ Press
- Number of pages
- 10
- Grant note
- INT15/00085 / Fondo de Investigaciones Sanitarias; Instituto de Salud Carlos III Hospital Clinic Barcelona (Ajut per a la Recerca Josep Font)
- Language
- English
- Date published
- 10/02/2021
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984354054002771
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