Results of a Nationwide Multicenter Study in Childhood Sjögren Disease.

Abstract

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.