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

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To characterize the phenotypic presentation at diagnosis of childhood-onset primary Sjögren syndrome (SjS).
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.
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.
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.


  • 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 Rasmussen
  • Pulukool 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
  • Sjögren Big Data Consortium
External organisations
  • Hospital CIMA
  • University of L'Aquila
  • Sapienza University of Rome
  • University of Pisa
  • University Medical Center Utrecht
  • Skåne University Hospital
  • Technical University of Munich
  • Federal University of São Paulo
  • University College London
  • Kanazawa University Hospital
  • University of Groningen
  • University of Barcelona
  • Polytechnic University of Catalonia
  • University Medical Center Groningen
  • University Medical Centre Ljubljana
  • Karolinska Institutet
  • Anhui Provincial Hospital
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Rheumatology and Autoimmunity
Original languageEnglish
Publication statusE-pub ahead of print - 2021 Jan 25
Publication categoryResearch