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

Sjogren Big Data Consortium, Manuel Ramos-Casals, Nihan Acar-Denizli, Arjan Vissink, Pilar Brito-Zerón, Xiaomei Li, Francesco Carubbi, Roberta Priori, Nataša Toplak, Chiara Baldini, Enrique Faugier-Fuentes, Aike A Kruize, Thomas Mandl, Minako Tomiita, Saviana Gandolfo, Kunio Hashimoto, Gabriela Hernandez-Molina, Benedikt Hofauer, Samara Mendieta-Zerón, Astrid RasmussenPulukool Sandhya, Damien Sene, Virginia Fernandes Moça Trevisani, David Isenberg, Erik Sundberg, Sandra G Pasoto, Agata Sebastian, Yasunori Suzuki, Soledad Retamozo, Bei Xu, Roberto Giacomelli, Angelica Gattamelata, Masa Bizjak, Stefano Bombardieri, Richard-Eduardo Loor-Chavez, Anneline Hinrichs, Peter Olsson, Hendrika Bootsma, Scott M Lieberman

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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 19years according to the fulfilment of the 2002/2016 classification criteria.

Results. Among the 12083 patients included in the Sjogren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2years): 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.

Original languageEnglish
Pages (from-to)4558-4567
Number of pages10
Issue number10
Early online date25-Jan-2021
Publication statusPublished - Oct-2021


  • Sjogren's syndrome
  • epidemiology
  • autoimmune diseases
  • paediatrics
  • childhood
  • AGE

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