PT  - JOURNAL ARTICLE
AU  - Kılbaş, Gülşah
AU  - Ayduran, Semra
AU  - Şener, Seher
AU  - Coşkuner, Taner
AU  - Ulu, Kadir
AU  - Kısaoğlu, Hakan
AU  - Aslan, Esma
AU  - Kılıç Könte, Elif
AU  - Arslanaoğlu, Ceyda
AU  - Aydın, Tuncay
AU  - Şadırvan Oğuzkaya, Yağmur
AU  - Çakmak, Figen
AU  - Gezgin Yıldırım, Deniz
AU  - Kaplan, Melike Mehveş
AU  - Tiğrak, Saadet Nilay
AU  - Türkuçar, Serkan
AU  - Sönmez, Hafize Emine
AU  - Kışla Ekinci, Miray
AU  - Öztürk, Kübra
AU  - Demir, Ferhat
AU  - Bağlan, Esra
AU  - Bozkaya, Burcu
AU  - Taşkın, Sema Nur
AU  - Demir, Selcan
AU  - Sağ, Erdal
AU  - Deniz Batu, Ezgi
AU  - Şahin, Sezgin
AU  - Bakkaloğlu Ezgü, Sevcan A.
AU  - Kılıc, Sara Sebnem
AU  - Paç Kısaarslan, Ayşenur
AU  - Çelikel Acar, Banu
AU  - Kalyoncu, Mukaddes
AU  - Ayaz, Nuray Aktay
AU  - Sözeri, Betül
AU  - Ünsal, Erbil
AU  - Kasapçopur, Özgür
AU  - Özen, Seza
AU  - Yüksel, Selçuk
TI  - Results of a Nationwide Multicenter Study in Childhood Sjögren Disease
AID  - 10.3899/jrheum.2024-1048
DP  - 2025 Jul 15
TA  - The Journal of Rheumatology
PG  - jrheum.2024-1048
4099  - http://www.jrheum.org/content/early/2025/07/09/jrheum.2024-1048.short
4100  - http://www.jrheum.org/content/early/2025/07/09/jrheum.2024-1048.full
AB  - Objective This nation-wide, multicenter study was conducted to assess the demographic, clinical features, treatment regimens, and prognosis of primary Sjögren disease (SjD) in childhood.Methods This retrospective study included a total of 81 patients under 18 years of age from 21 pediatric rheumatology centers. Among these, 51 patients fulfilled the diagnosis of childhood SjD strictly according to the 2016 ACR/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 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, while the remaining 30 were classified as an at-risk group. The most common clinical findings in 30 patients '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, p = 0.02, respectively).Conclusion The current classification criteria for childhood SjD appear to be inadequate, highlighting the need for pediatric-specific criteria that more accurately reflect the distinct clinical patterns observed in children.