Pediatric Sjögren Syndrome: Case Report On A Rare Entity

Area of Science:

• Autoimmune diseases
• Pediatric rheumatology
• Immunology

Background:

• Sjögren's Syndrome (SS) is an autoimmune disorder with unknown prevalence and high comorbidity risk.
• Pediatric SS often presents with recurrent parotiditis and sialadenitis, unlike adult symptoms of dry eyes and mouth.
• Early diagnosis and management are crucial for improving outcomes in pediatric autoimmune conditions.

Observation:

• A 16-year-old girl presented with recurrent cervical lymphadenopathy and parotid swelling.
• Extensive investigations over nine months revealed chronic bilateral sialadenitis of unknown etiology.
• Initial presentation suggested primary SS, but the patient later met criteria for Systemic Lupus Erythematosus.

Findings:

• The case highlights diagnostic challenges in pediatric Sjögren's Syndrome.
• Co-occurrence of SS and Systemic Lupus Erythematosus in a pediatric patient.
• Successful management with hydroxychloroquine, but long-term follow-up is essential.

Implications:

• Emphasizes the need for specific diagnostic criteria for pediatric Sjögren's Syndrome.
• Underscores the importance of multidisciplinary care for complex autoimmune cases in children.
• Suggests potential overlap and diagnostic complexities between pediatric SS and other autoimmune diseases like lupus.