Pediatric Sjögren Syndrome: Case Report On A Rare Entity

Pediatric Sjögren's Syndrome (SS) is an auto-immune disorder of unknown prevalence with significant risk of comorbidity. In contrast to the classical dyad of xerostomia and xeropthalmia frequently seen in adults, in children and adolescents, recurrent parotiditis and sialadenitis are more often the presenting symptoms. We describe the case of a previously healthy 16-year-old girl with recurrent cervical lymphadenopathy and parotid swelling. Over the course of nine months, extensive investigation established chronic bilateral recurrent sialadenitis of unknown cause. The patient's clinic and complementary exams favor a primary SS diagnosis; however, she later meets classification criteria for Systemic Lupus Erythematosus. Although currently clinically stable under hydroxychloroquine with minor parotid swelling and eye redness, long term multidisciplinary follow-up will be needed to manage the patient's disease. This report aims to bring awareness to this diagnostic challenge and to the need for pediatric criteria for SS.