Sjögren's syndrome is an autoimmune condition characterized by periductal and perivascular lymphocytic infiltration of exocrine glandular ducts, xerostomia, and keratoconjunctivitis sicca. It was first described by Henrik Sjögren and Henri Gougerot in the early 1900's. Since that time, Sjögren's syndrome has been relatively underreported in pediatric literature. Although similar to the disease in the adult population, pediatric Sjögren's most notably presents with less sicca and more parotid swelling. In this chapter, we will describe the unique clinical, serological, and extraglandular manifestations of Sjögren's syndrome in the pediatric population. Furthermore, we will review the diagnostic and therapeutic approach by evaluating the risks and benefits of pursuing an invasive diagnostic workup in a young population, as well as the implications of treatment that can sometimes require long-term immunosuppression.
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