Sjögren's syndrome (SS) in childhood: is it essentially different from adult SS?

Abstract

  Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by a progressive lymphocytic infiltration of the exocrine glands with varying degrees of systemic involvement. Chronic inflammation compromised the glands' function and leads to dry symptoms in the mouth/eyes. SS has been thought to be rare in children. Recent epidemiological study revealed, however, that the incident of childhood SS (cSS) per 100,000 children was more than 0.5. This indicate cSS is common disease after SLE in children with rheumatic diseases. Sicca symptoms are rare in cSS even though oral and ocular involvement are present. This may be from the slowly progression feature of the disease course. Two follow-up studies indicate that there were almost no changes in clinical symptoms and autoantibody profiles during the 10 years of follow-up. However, auto-antibody profiles of cSS including alpha-fodrin-antibody, specifically found in adult SS, are the same as that of adult SS. These data indicate that no essential difference exist in SS between adult and childhood patients, and also indicate the specific features seen in cCSS just reflect early stage of SS. In order to establish the diagnostic criteria for cSS in the feature, early pathophysiologic features should be included in the criteria.