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Published on: October 13, 2023
Paediatric Sjögren's Syndrome With Bilateral Parotid Cysts: A Case Report
Jamila Skinner1, James Fowler1, Jonathan Park2
1Department of Otolaryngology - Head and Neck Surgery, London Health Sciences Centre - Victoria Hospital, London, CAN.
Insights
Sjögren's syndrome in children can present unusually with parotid gland cysts, not just dry eyes or mouth. Early diagnosis requires considering rare symptoms like bilateral parotid masses.
Area of Science:
- Autoimmune diseases
- Pediatric Rheumatology
- Ophthalmology
Background:
- Sjögren's syndrome is an autoimmune disorder causing exocrine gland destruction.
- Adults typically present with dry eyes (xerophthalmia) and dry mouth (xerostomia).
- Pediatric patients often exhibit recurrent parotitis and glandular enlargement, complicating diagnosis.
Observation:
- A 12-year-old female presented with bilateral parotid masses over two months.
- She denied typical sicca symptoms but imaging revealed complex cystic intraparotid masses.
- Biopsy showed dense lymphoplasmacytic infiltrate in the parotid gland.
Findings:
- The case highlights rare parotid cysts as a presentation of Sjögren's syndrome in a pediatric patient.
- This presentation deviated from the common sicca symptoms seen in adults.
- Dense lymphoplasmacytic infiltrate confirmed autoimmune activity.
Implications:
- This case underscores the need for comprehensive diagnostic evaluation in pediatric Sjögren's syndrome.
- Parotid cysts should be included in the differential diagnosis for pediatric parotid swelling.
- Recognizing atypical presentations is crucial for timely Sjögren's syndrome diagnosis in children.
Abstract:
Sjögren's syndrome is an autoimmune disease characterized by the destruction of exocrine glands. Clinically, this results in the loss of tear and saliva production. Although xerophthalmia and xerostomia, also known as sicca, is a common presentation among adults, paediatric patients more often present with recurrent parotitis and glandular enlargement. Overall, symptoms can vary, making initial diagnosis challenging. Approximately 80% of patients with Sjögren's syndrome experience parotid gland enlargement, however, salivary cysts are rare. Herein, we present a case of paediatric Sjögren's syndrome where a 12-year-old female presented with a two-month history of bilateral parotid masses. The patient denied any history of xerostomia, xerophthalmia, or constitutional symptoms. Imaging revealed bilateral complex cystic intraparotid masses. A right parotid gland biopsy was performed showing parotid gland parenchyma with dense lymphoplasmacytic infiltrate. Ultimately, the presumptive diagnosis of Sjögren's syndrome was made. This case illustrates the importance of a thorough workup to aid in diagnostic certainty. Parotid cysts associated with Sjögren's are rare but should be considered within the differential diagnosis for paediatric patients with parotid swelling/mass.
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