The clinical presentation of childhood Sjögren’s widely varies from child to child. Unlike adults, the most common presentation in children is parotid enlargement. Many children have aspects of ocular and oral dryness at presentation but have difficulty perceiving and reporting these symptoms as dryness. Vaginal irritation can occur affecting around 5% of the females with childhood Sjögren’s. Joint symptoms, a characteristic rash, fatigue, fevers, and enlarged lymph nodes also occur. Neurologic, kidney, blood, lungs, and abdominal manifestations can occur but are more rare presentations.
There are no valid diagnostic criteria specific for childhood Sjögren’s. Many aspects of the adult criteria are difficult to complete and interpret in children. Therefore, diagnosing childhood Sjögren’s can be difficult and is based on multiple clinical features as well as clinical judgement of the treating physician.