RT Journal Article
SR Electronic
T1 Results of a Nationwide Multicenter Study in Childhood Sjögren Disease
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP jrheum.2024-1048
DO 10.3899/jrheum.2024-1048
A1 Kılbaş, Gülşah
A1 Ayduran, Semra
A1 Şener, Seher
A1 Coşkuner, Taner
A1 Ulu, Kadir
A1 Kısaoğlu, Hakan
A1 Aslan, Esma
A1 Kılıç Könte, Elif
A1 Arslanaoğlu, Ceyda
A1 Aydın, Tuncay
A1 Şadırvan Oğuzkaya, Yağmur
A1 Çakmak, Figen
A1 Gezgin Yıldırım, Deniz
A1 Kaplan, Melike Mehveş
A1 Tiğrak, Saadet Nilay
A1 Türkuçar, Serkan
A1 Sönmez, Hafize Emine
A1 Kışla Ekinci, Miray
A1 Öztürk, Kübra
A1 Demir, Ferhat
A1 Bağlan, Esra
A1 Bozkaya, Burcu
A1 Taşkın, Sema Nur
A1 Demir, Selcan
A1 Sağ, Erdal
A1 Deniz Batu, Ezgi
A1 Şahin, Sezgin
A1 Bakkaloğlu Ezgü, Sevcan A.
A1 Kılıc, Sara Sebnem
A1 Paç Kısaarslan, Ayşenur
A1 Çelikel Acar, Banu
A1 Kalyoncu, Mukaddes
A1 Ayaz, Nuray Aktay
A1 Sözeri, Betül
A1 Ünsal, Erbil
A1 Kasapçopur, Özgür
A1 Özen, Seza
A1 Yüksel, Selçuk
YR 2025
UL http://www.jrheum.org/content/early/2025/07/09/jrheum.2024-1048.abstract
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.