DOI: 10.1177/00494755261503706 ISSN: 0049-4755

Diagnostic odyssey of juvenile onset primary Sjögren's disease: Recurrent neck swelling and rampant dental caries in a 17-year old female

Abhijeet Dhanshetty, Deepthi B, Ram Rapur

Primary Sjögren's disease is an exceedingly rare auto-immune disorder in paediatric and adolescent populations, frequently leading to prolonged diagnostic delays due to atypical initial presentations. We report a 17-year old female presenting with a four-year history of recurrent neck swelling, dysphagia, rampant dental caries and progressive xerostomia, previously misdiagnosed and managed empirically as viral infections and reactive lymphadenitis. Physical examination confirmed severe oral dryness and hyposalivation. Serological testing revealed positive antinuclear antibodies with high titers of anti-Ro (SSA) and anti-La (SSB). A labial minor salivary gland excision biopsy demonstrated focal lymphocytic sialadenitis, definitively confirming juvenile-onset primary Sjögren's disease.

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