Abstract
Sjögren syndrome is a heterogeneous chronic multisystem autoimmune rheumatic condition, with manifestations ranging from localized glandular disease to systemic and organ system involvement. This disease has been considered to be rare in children and adolescents; however, it has almost certainly been underdiagnosed and is more common than previously realized. This chapter reviews the epidemiology of this condition in childhood, the spectrum of clinical manifestations including those newly recognized as being associated with Sjögren syndrome, the pathophysiology, and the newest treatment recommendations.
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