Journal article
Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria
Rheumatology (Oxford, England), Vol.60(7), pp.3144-3155
12/06/2020
DOI: 10.1093/rheumatology/keaa757
PMCID: PMC8487648
PMID: 33280020
Abstract
Objective: Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population. Methods: An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations. We analysed patterns of symptoms, diagnostic workup, and applied the 2016 ACR/EULAR classification criteria. Results: We identified 300 children with Sjögren syndrome. The majority of patients n = 232 (77%) did not meet 2016 ACR/EULAR classification criteria, but n = 110 (37%) did not have sufficient testing done to even possibly achieve the score necessary to meet criteria. Even among those children with all criteria items tested, only 36% met criteria. The most common non-sicca symptoms were arthralgia [n = 161 (54%)] and parotitis [n = 140 (47%)] with parotitis inversely correlating with age. Conclusion: Sjögren syndrome in children can present at any age. Recurrent or persistent parotitis and arthralgias are common symptoms that should prompt clinicians to consider the possibility of Sjögren syndrome. The majority of children diagnosed with Sjögren syndromes did not meet 2016 ACR/EULAR classification criteria. Comprehensive diagnostic testing from the 2016 ACR/EULAR criteria are not universally performed. This may lead to under-recognition and emphasizes a need for further research including creation of paediatric-specific classification criteria.
Details
- Title: Subtitle
- Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria
- Creators
- Matthew L Basiaga - Mayo Clinic in FloridaSara M Stern - University of UtahJay J Mehta - Children's Hospital of PhiladelphiaCuoghi Edens - University of Chicago Medical CenterRachel L Randell - Duke UniversityAnna Pomorska - Gdańsk Medical UniversityNinela Irga-Jaworska - Gdańsk Medical UniversityMaria F Ibarra - University of Missouri–Kansas CityClaudia Bracaglia - Bambino Gesù Children's HospitalRebecca Nicolai - Bambino Gesù Children's HospitalGordana Susic - Center for RheumatologyAlexis Boneparth - Columbia University Irving Medical CenterHemalatha Srinivasalu - George Washington UniversityBrian Dizon - George Washington UniversityAnkur A Kamdar - The University of Texas Health Science Center at HoustonBaruch Goldberg - Children's Healthcare of AtlantaSheila Knupp-Oliveira - Universidade Federal do Rio de JaneiroJordi Antón - Universitat de BarcelonaJuan M Mosquera - Hospital Sant Joan de Déu BarcelonaSimone Appenzeller - Universidade Estadual de Campinas (UNICAMP)Kathleen M O’Neil - Indiana University HealthStella A Protopapas - Riley Hospital for ChildrenClaudia Saad-Magalhães - Universidade Estadual Paulista (Unesp)Jonathan D Akikusa - Royal Children's HospitalAkaluck Thatayatikom - University of FloridaSeunghee Cha - University of FloridaJuan Carlos Nieto-González - Hospital General Universitario Gregorio MarañónMindy S Lo - Boston Children's HospitalErin Brennan Treemarcki - University of UtahNaoto Yokogawa - Tokyo Metropolitan Tama Medical CenterScott M Lieberman - University of IowaChildhood Arthritis and Rheumatology Research Alliance InvestigatorsInternational Childhood Sjögren Syndrome Workgroup
- Resource Type
- Journal article
- Publication Details
- Rheumatology (Oxford, England), Vol.60(7), pp.3144-3155
- Publisher
- Oxford University Press
- DOI
- 10.1093/rheumatology/keaa757
- PMID
- 33280020
- PMCID
- PMC8487648
- ISSN
- 1462-0324
- eISSN
- 1462-0332
- Grant note
- ;
- Language
- English
- Date published
- 12/06/2020
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984353928802771
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