Results of a Nationwide Multicenter Study in Childhood Sjögren Disease

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dc.authorid0000-0001-9265-288X
dc.authorid0000-0002-6183-0935
dc.authorid0000-0002-1800-9922
dc.authorid0000-0002-1125-7720
dc.authorid0000-0003-1564-8996
dc.authorid0000-0003-0381-6040
dc.authorid0000-0002-8197-6077
dc.contributor.authorKilbas, Gulsah
dc.contributor.authorAyduran, Semra
dc.contributor.authorSener, Seher
dc.contributor.authorCoskuner, Taner
dc.contributor.authorUlu, Kadir
dc.contributor.authorKisaoglu, Hakan
dc.contributor.authorAslan, Esma
dc.date.accessioned2026-02-03T11:59:46Z
dc.date.available2026-02-03T11:59:46Z
dc.date.issued2025
dc.departmentÇanakkale Onsekiz Mart Üniversitesi
dc.description.abstractObjective. This nationwide, multicenter study was conducted to assess the demographic and clinical features, treatment regimens, and prognosis of primary Sj & ouml;gren disease (SjD) in childhood. Methods. This retrospective study included a total of 81 patients < 18 years of age from 21 pediatric rheumatology centers. Among these, 51 patients fulfilled the diagnosis of childhood SjD according to the 2016 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria. The remaining 30 patients-who did not fully meet these criteria but exhibited clinical and laboratory findings suggestive of SjD-were categorized as at risk for childhood SjD to highlight diagnostic challenges and the spectrum of early presentations, based on comprehensive clinical evaluation by experienced pediatric rheumatologists. Results. The cohort consisted of 81 patients (85.2% female, 14.8% male) with a median age at symptom onset of 11.4 years and a median follow-up of 24 months. Common clinical manifestations included dry mouth, dry eyes, arthralgia, fatigue, and parotitis. Fifty-one of the 81 patients met the 2016 ACR/EULAR classification criteria, whereas the remaining 30 were classified as an at-risk group. The most common clinical findings in the at-risk group were xerostomia (90%), arthralgia (56.7%), fatigue (50%), and dry eyes (43.3%). Dry mouth and peripheral nervous system involvements were found to be higher in patients categorized as at risk (P = 0.03 and P = 0.02, respectively). Conclusion. The current classification criteria for childhood SjD appear to be inadequate, highlighting the need forpediatric-specific criteria that more accurately reflect the distinct clinical patterns observed in children.
dc.identifier.doi10.3899/jrheum.2024-1048
dc.identifier.endpage1150
dc.identifier.issn0315-162X
dc.identifier.issn1499-2752
dc.identifier.issue11
dc.identifier.pmid40664482
dc.identifier.startpage1141
dc.identifier.urihttps://doi.org/10.3899/jrheum.2024-1048
dc.identifier.urihttps://hdl.handle.net/20.500.12428/34413
dc.identifier.volume52
dc.identifier.wosWOS:001620464600011
dc.identifier.wosqualityQ2
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherJ Rheumatol Publ Co
dc.relation.ispartofJournal of Rheumatology
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.snmzKA_WOS_20260130
dc.subjectautoimmune diseases
dc.subjectchildhood
dc.subjectparotitis
dc.subjectSj & ouml;gren disease
dc.titleResults of a Nationwide Multicenter Study in Childhood Sjögren Disease
dc.typeArticle

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