Sjögren's syndrome is uncommon in children, and occurs most often in association with autoimmune diseases (secondary Sjögren's syndrome). We describe the clinical and biological features of a 13-year-old girl with primary Sjögren's syndrome, revealed by recurrent parotitis.
This adolescent girl was referred for investigation of multiple episodes of bilateral parotid swelling since age nine, without systemic symptoms. Examination was unremarkable except for enlarged and painless parotid glands. Laboratory investigations, measurement of saliva production, parotid sialography, labial salivary gland biopsy, revealed Sjögren's syndrome without associated disease. Hydroxychloroquine was prescribed with clinical improvement.
Recurrent parotitis in children is an uncommon condition. The onset of parotid swelling at five years or over deserves screening for dysimmune disorders, sarcoidosis or Sjögren's syndrome. Diagnosis of Sjögren's syndrome is based on laboratory evidence of autoimmune disorders and minor salivary gland biopsy.