Unique Aspects of Pediatric Sjögren Disease

Abstract

Sjögren disease increasingly is recognized in pediatric patients. Clinical features, primarily parotitis and sicca symptoms, and results of diagnostic tests may be different from those in adult disease. Adult criteria fail to capture most pediatric patients. Pediatric-specific criteria are urgently needed to define the natural history of the disease, identify risk and prognostic factors, and evaluate the impact of therapeutics and other interventions on disease course in young patients.

Keywords: Parotitis; Pediatric Sjögren syndrome; Sjögren disease; Sjögren syndrome.

Figure 1.

Focal sialadenitis on labial minor salivary gland biopsy in a child with Sjögren disease. This H&E-stained section of a minor salivary gland from an 8-year-old girl demonstrates normal glandular architecture with a focus of mononuclear cells (arrow) in the typical peri-ductal distribution. A focus is defined as an aggregate of ≥50 mononuclear cells. The focus score is defined as the number of foci per 4 mm² of tissue, with a score of ≥1 focus/4 mm² meeting histopathologic criteria for Sjögren disease in adults. Courtesy of Scott Steward-Tharp, DDS, PhD, University of Iowa.