ArticleRheumatology (Oxford, England)2021
Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria.
Article in Rheumatology (Oxford, England), 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers, 2 of them syntheses that pooled it.
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Who cites it
38 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease.Rheumatology (Oxford, England) · 2025Guideline
- Treatment strategies for Sjögren's syndrome with childhood onset: a systematic review of the literature.Rheumatology (Oxford, England) · 2022Pooled it
- The Diagnostic Performance of Early Sjögren's Syndrome Autoantibodies in Juvenile Sjögren's Syndrome: The University of Florida Pediatric Cohort Study.Frontiers in immunology · 2021Trial
- Managing Sjögren's Disease in Pediatric Patients.Paediatric drugs · 2026Review
- Minor salivary gland biopsies in childhood-onset Sjögren's disease show greater inflammation and more frequent fibrotic changes than in adult-onset Sjögren's disease.Arthritis research & therapy · 2026Article
- Pediatric Sjögren's Disease: Literature Review and Diagnostic Challenges in an Uncommon Case.Diagnostics (Basel, Switzerland) · 2026Article
- Pediatric Sjögren Disease: Clinical Features, Diagnostic Challenges, and Outcomes in a Single-Centre Romanian Case Series.Journal of clinical medicine · 2026Article
- Hormonal transitions across the lifespan shape susceptibility to Sjogren's disease.Rheumatology (Oxford, England) · 2026Article
- Case Report: Autoimmune-associated tubulointerstitial nephritis with predominant Sjögren features in a child presenting with recurrent sterile pyuria.Frontiers in pediatrics · 2026Article
- Childhood Sjögren's Disease: A Literature Review of an Underrecognized Autoimmune Entity in Pediatric Rheumatology.Cureus · 2025Review
- Childhood-onset primary Sjögren's Syndrome presenting as nephrotic syndrome: a case report and literature review.BMC pediatrics · 2025Review
- Diminished SUV3 expression and its functional implications in the IFN-enriched monocyte subset of childhood Sjögren's disease.Rheumatology (Oxford, England) · 2025Article
- Pediatric Sjögren's Syndrome: Focus on Ocular Involvement and Diagnostic Challenges.Medicina (Kaunas, Lithuania) · 2025Article
- Do We Need Distinct Pediatric Classification Criteria for Rheumatic Diseases That Affect Both Children and Adults?Arthritis & rheumatology (Hoboken, N.J.) · 2025Article
- Neuropsychiatric manifestations in systemic lupus erythematosus and Sjogren's disease.Autoimmunity reviews · 2025Review
- Article
- Mechanisms underlying sex differences in autoimmunity.The Journal of clinical investigation · 2024Review
- More severe parotid gland histopathology in paediatric-onset than in adult-onset Sjögren's disease.RMD open · 2024Article
- Spectrum of Central Nervous System Involvement in Childhood-Onset Sjögren's Syndrome: A Case-Based Review.Mediterranean journal of rheumatology · 2024Article
- The Florida Scoring System for stratifying children with suspected Sjögren's disease: a cross-sectional machine learning study.The Lancet. Rheumatology · 2024Article
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Authors and funding
32 authors.
Funding
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Abstract
objectiveSjögren 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.
methodsAn 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.
resultsWe 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.
conclusionSjögren 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.
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