[Primary Sjögren syndrome in a child]

Area of Science:

• Pediatrics
• Rheumatology
• Immunology

Background:

• Sjögren syndrome is typically diagnosed in adults and often secondary to other autoimmune conditions.
• Primary Sjögren syndrome in pediatric populations is uncommon and presents diagnostic challenges.

Observation:

• A 7-year-old girl experienced recurrent, painless parotid gland swelling starting at age 4.
• Initial presentation lacked systemic symptoms, with examination revealing only enlarged parotid glands.

Findings:

• Diagnostic workup, including labial salivary gland biopsy, confirmed primary Sjögren syndrome.
• The patient showed clinical improvement after treatment with hydroxychloroquine.

Implications:

• Recurrent parotiditis in children over age 5 warrants investigation for autoimmune diseases like Sjögren syndrome.
• Adherence to diagnostic criteria is crucial for accurate Sjögren syndrome diagnosis in pediatric cases.
• Early diagnosis and management can improve outcomes for children with primary Sjögren syndrome.