Sjögren's syndrome in childhood

This review presents our 10-year experience with children diagnosed with Sjögren's syndrome (SS). Patients between the ages of 9 and 17 years had abnormalities in laboratory values consistent with but not entirely diagnostic of those required to diagnose SS in adults. The spectrum of clinical manifestations suggests that the SS clinical phenotype in children is more variable than that in adults. Here, we review manifestations of SS in children. Our patients were treated with hydroxychloroquine, despite the lack of prospective data about effects on SS progression and/or autoantibody spreading. Patients have been followed for between 3 and 6 years without substantial progression of their disease or change in autoantibody status. Longer term follow-up (10-20 years) is needed to define the natural history of SS in childhood and its treatment outcomes. Prospective validation of SS criteria in childhood could facilitate assessment of the utility of hydroxychloroquine and other therapies.