AB0944 SJöGREN’S SYNDROME IN CHILDREN: A CASE SERIES

Abstract

Background The symptoms of pediatric Sjogren’s syndrome (SS) are different than in adults. There are currently no validated pediatric diagnostic criteria or treatment guidelines for SS. In most cases adult criteria are used, but they apply poorly to children. Objectives To present pediatric patients with primary SS who were treated at University Children’s Hospital (UCH) Ljubljana in the past 10 years. Methods Eight children with primary SS were identified. Demographic data, clinical and laboratory findings and therapy were analysed by retrospective review of medical records at UCH Ljubljana. Results Six girls and 2 boys were evaluated. The mean age at disease onset was 12.3 years (range 6.5 – 17) and mean age at diagnosis was 13.8 years (range 7.5 – 17.5). The mean follow-up duration was 2.8 years (range 0.5 – 8.5). Four patients presented with recurrent bilateral parotitis, two with rash, one with arthralgia and fatigue and one with acute central nervous system vasculitis. The latter patient presented with rheumatic fever at the same time. During disease course arthritis and/or arthralgia was present in 5/8, parotitis in 4/8, oral symptoms in 4/8, rash in 4/8, fatigue in 3/8 and ocular symptoms in 2/8 patients. One patient developed calcinations on fingers. All patients had high titer of antinuclear antibodies, 7/8 were positive for anti-Ro and 5/8 for anti-La antibodies. Three patients were tested for rheumatoid factor and all were positive. 6/8 patients had elevated ESR and hypergammaglobulinemia. 4/8 patients had elevated serum amylase. Biopsy of salivary glands was performed in 5 patients and foci of lymphocytic infiltration were shown in all of them. Further two patients had salivary gland changes on MRI and/or US. On ophthalmologic evaluation 3 patients had positive Schirmer test and one had unstable tear film. One patient had CNS vasculitis and two decreased diffusing capacity of the lungs. Other patients showed no signs of internal organ involvement. 4/8 patients were treated with NSAIDs, 7/8 patients with hydroxychloroquine, 2/8 also received corticosteroids and one patient MMF. At this point the patient with CNS vasculitis has stable changes on head MRI without clinical symptoms. Other patients have no signs of internal organ damage. Calcinations that appeared on fingers in one patient are not progressing. Conclusion Most common symptoms in our cohort were arthritis and/or arthralgia, parotitis, oral symptoms and rash. Half of the children presented with recurrent parotitis. One child developed calcinations on fingers, which have not yet been described in patients with SS. Pediatric SS differs from adult SS and specific pediatric diagnostic criteria would improve management of these patients.