Sjögren's Syndrome: A Rare Clinical Entity in Children

Abstract

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.