Juvenile Sjogren's Syndrome

Sjogren's syndrome in adolescents is a rare autoimmune disease, which is often diagnosed with delay. It may present as a primary juvenile Sjogren's syndrome (jSS) or in association with other autoimmune diseases as a secondary form of jSS. Differential diagnoses include other forms of parotitis, which are more frequent in that age group. The clinical presentation of jSS differs from SS in adults in several aspects. However, severe systemic illness and even the development of B-cell lymphoma have also been observed in young patients suffering from jSS. Therefore, jSS should be considered as a diagnosis in cases of recurrent sialadenitis in adolescents if there are other suspicious findings. Early diagnosis as well as tight monitoring and treatment by paediatric rheumatologists are mandatory in patients with jSS in order to control disease activity and to minimise long-term damage.