O20 A case series of paediatric primary Sjögren’s syndrome: differential diagnoses and multidisciplinary management

Abstract Case report - Introduction Two cases of primary Sjögren’s syndrome presenting in a child with recurrent salivary gland enlargement are described. As a condition which rarely presents in childhood, these cases underline the need to consider more uncommon differentials in a child presenting with recurrent salivary gland swellings. They also highlight how well co-ordinated multidisciplinary care involving medical and dental specialist teams can achieve the best outcomes for patients. Case report - Case description Case 1: An 8-year-old girl presented to Paediatric Rheumatology with non-mealtime related episodes of bilateral parotid pain and swelling and intermittent dry mouth. Dry eyes and joint pain were not reported, and she was systemically well. Anti-Ro, anti-La and rheumatoid factor were positive. Salivary gland ultrasound showed evidence of sialadenitis. Sjögren’s Syndrome was diagnosed and treatment with hydroxychloroquine and azathioprine reduced the swelling frequency and severity. Regular optician reviews were arranged, and she was also noted to have ‘soft teeth’. Review by Paediatric Dentistry revealed her dry mouth had been managed with acidic sweets, leading to erosive tooth surface loss of the primary dentition. Onward referral was made to Oral Medicine and a differential diagnosis of chronic parotitis of childhood was considered. Ultrasound review by Oral and Maxillofacial Radiology confirmed Sjögren’s Syndrome. Yearly ultrasound scanning was arranged to assess for disease progression and lymphoma development. Case 2: A 17-year-old girl was referred to Oral Medicine with a diagnosis of Sjögren’s syndrome. She initially presented to Rheumatology 3 years previously with dry mouth, dry eyes, and intermittent bilateral swellings of the parotid and submandibular glands. The salivary gland swellings were associated with pain on eating and fatigue and improved with short courses of oral corticosteroids. Titres of anti-Ro60/52 antibodies were high, and a right parotid ultrasound guided biopsy showed a focus score of greater than 2 with changes consistent with Sjögren’s Syndrome. Hydroxychloroquine was initiated and the frequency and severity of the swellings improved. Referrals were made to Ophthalmology for a dry eye’s ocular assessment and retinal screening in view of hydroxychloroquine therapy. She regularly attended her dentist and managed her dry mouth with sugar free sweets. On review with Oral Medicine various non-acidic oral lubricants