Sjogren's syndrome in childhood

Objective. To describe the clinical characteristics of juvenile Sjogren's syndrome (JSS) and report 5 new primary cases. Methods. Patients with SS whose disease began before age 16 were identified from a cohort study on SS. Previons patients with JSS published from 1952 to 1993 were found by literature review. Thirty-nine adult patients with primary SS were selected as a control group. Results. Five patients with primary JSS were identified and described. Thirty-four published primary JSS were reviewed. Altogether, there were 30 girls (77%). The mean age at onset was 7.8 ± 4 years. Parotitis was the most common first symptom. An extraglandular manifestation (EGM) was the presenting feature in 9.3% of cases. Rheumatoid factor (RF) was positive in 71% and antinuclear antibodies (ANA) in 67%. During the course of disease, at least one EGM was noted in 20 cases (51%). Leukopenia was the most frequent of these (7/20, 35%). HLA-DR3 was observed in 4 of 6 cases in which HLA typing was done. Compared with adult primary SS, parotitis at onset was more frequent in children (62.5 vs 13%). The frequency of ANA and articular manifestations were higher in adults (67 vs 92%, and 15.4 vs 38.5%, respectively). We reviewed 31 cases of secondary JSS, of which the most common associated autoimmune disease was juvenile rheumatoid arthritis (42%). JSS preceded by years the associated autoimmune disease in nearly 50% of cases. Conclusion. JSS may be a common disease. Parotitis and the presence of RF and ANA are the main features at onset. Characteristics similar to those seen in adults are observed in children. Longterm followup is needed to assess outcome associated factors.