Background/Purpose: Sjögren’s syndrome is a chronic autoimmune disease that involved the exocrine glands, and primary Sjögren’s syndrome occurs rarely in children and adolescents. It is known that exocrine gland invasions such as dry eye or dry mouth decrease quality of life and increase the risk of malignancy in long term outcomes, so regular follow-up is necessary. Therefore, we aimed to identify in clinical features of primary childhood Sjögren’s syndrome in South Korea to help with diagnosis and treatment.

Methods: We retrospectively analyzed with childhood-onset primary Sjögren’s syndrome underage of 20 years from 2008 to 2024. Data from patients who occurred at Seoul National University Children’s Hospital and Pusan National University Children’s Hospital were collected through electronic medical records.

Results: A total of 20 patients, comprising 5 males and 15 females, exhibited a sex ratio of 1:3. The age at diagnosis was 14.2±2.5 years, and the age at last follow-up was 18.3±4.0 years. The total observation period was 48.0±42.1 months. Among the accompanying symptoms at the time of diagnosis, inflammatory reactions such as fever were the most common at 70%, followed by sialadenitis at 45%, and dry mouth, musculoskeletal involvement, and skin involvement at 40% respectively. In the early-onset group, parotitis was a prevalent symptom, whereas xerophthalmia was more common in the late-onset group. All patients fulfilled the 1999 proposed criteria for jSS and 2018 Japanese criteria, but only 15% met the 2016 ACR/EULAR criteria. Pharmacological treatment was administered to 85% of patients, with hydroxychloroquine and corticosteroids bed the primary medication used. At the last follow-up, improvements in inflammatory markers and ESSDAI scores were observed, with the initial low-ESSDAI group demonstrating lower final ESSDAI scores. During the follow-up period, one patients was diagnosed with dermatofibrosarcoma.

Conclusion: The clinical symptoms of childhood-onset primary Sjögren’s syndrome are different from those in adults. Even if they do not initially meet the diagnostic criteria, regular follow-up was necessary, and appropriate treatment should be provided. Additional multicenter studies will be important to understand the characteristics of childhood-onset primary Sjögren’s syndrome.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-characteristics-of-childhood-onset-primary-sjogrens-syndrome-in-south-korea-two-centers-experiences/