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

Ramos-Casals, Manuel ; Acar-Denizli, Nihan ; Vissink, Arjan ; Brito-Zerón, Pilar ; Li, Xiaomei ; Carubbi, Francesco ; Priori, Roberta ; Toplak, Nataša ; Baldini, Chiara and Faugier-fuentes, Enrique , et al. (2021) In Rheumatology 60(10). p.4558-4567
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... (More)
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. (Less)
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author collaboration
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Rheumatology
volume
60
issue
10
pages
4558 - 4567
publisher
Oxford University Press
external identifiers
  • pmid:33493333
  • scopus:85113866014
ISSN
1462-0324
DOI
10.1093/rheumatology/keab032
language
English
LU publication?
yes
id
907622f3-1248-4f21-b2dd-0b9f72080782
date added to LUP
2021-01-26 07:45:31
date last changed
2022-04-26 23:56:04
@article{907622f3-1248-4f21-b2dd-0b9f72080782,
  abstract     = {{OBJECTIVES<br/>To characterize the phenotypic presentation at diagnosis of childhood-onset primary Sjögren syndrome (SjS).<br/>METHODS<br/>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.<br/>RESULTS<br/>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.<br/>CONCLUSIONS<br/>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.}},
  author       = {{Ramos-Casals, Manuel and Acar-Denizli, Nihan and Vissink, Arjan and Brito-Zerón, Pilar and Li, Xiaomei and Carubbi, Francesco and Priori, Roberta and Toplak, Nataša and Baldini, Chiara and Faugier-fuentes, Enrique and Kruize, Aike A and Mandl, Thomas and Tomiita, Minako and Gandolfo, Saviana and Hashimoto, Kunio and Hernandez-Molina, Gabriela and Hofauer, Benedikt and Mendieta-Zerón, Samara and Rasmussen, Astrid and Sandhya, Pulukool and Sene, Damien and Trevisani, Virginia Fernandes Moça and Isenberg, David and Sundberg, Erik and Pasoto, Sandra G and Sebastian, Agata and Suzuki, Yasunori and Retamozo, Soledad and Xu, Bei and Giacomelli, Roberto and Gattamelata, Angelica and Bizjak, Masa and Bombardieri, Stefano and Loor-chavez, Richard-eduardo and Hinrichs, Anneline and Olsson, Peter and Bootsma, Hendrika and Lieberman, Scott M}},
  issn         = {{1462-0324}},
  language     = {{eng}},
  number       = {{10}},
  pages        = {{4558--4567}},
  publisher    = {{Oxford University Press}},
  series       = {{Rheumatology}},
  title        = {{Childhood-onset of primary Sjögren’s syndrome: phenotypic characterization at diagnosis of 158 children}},
  url          = {{http://dx.doi.org/10.1093/rheumatology/keab032}},
  doi          = {{10.1093/rheumatology/keab032}},
  volume       = {{60}},
  year         = {{2021}},
}