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Background. Sjögren's syndrome (SS) is a rare autoimmune disease in children, with only a few hundred reported cases. Its clinical presentation is atypical. We report the case of a 14-year-old boy diagnosed with recurrent parotitis.

Case Presentation. Our patient, a 14-year-old male, presented with a one-year history of recurrent bilateral parotid swellings. Clinical examination revealed an objective oculo-buccal dryness. Ultrasound imaging showed hypoechoic hypertrophy of the parotid glands. Anti-SSA/SSB antibodies were strongly positive. Histological study of the accessory salivary glands revealed lymphocytic sialadenitis with a focus score of 3. The diagnosis of SS was confirmed according to the ACR/EULAR 2016 criteria. The combination of treatment with hydroxychloroquine and corticosteroids resulted in the resolution of clinical signs and biological inflammatory markers.

Conclusion. Given the rarity of SS in children, several diagnostic and therapeutic difficulties arise, in the absence of classification criteria adapted to the juvenile form of the disease, and the absence of therapeutic recommendations consolidated by clinical trials in children.

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How to Cite
Hamri, L., Saoudi, S., & Rabhi, S. (2023). Sjögren’s Syndrome: A Rare Clinical Entity in Children. Indonesian Journal of Rheumatology, 15(2), 786-790.