SJOGRENS-SYNDROME IN CHILDHOOD

Objective. To describe the clinical characteristics of juvenile Sjogre n's syndrome (JSS) and report 5 new primary cases. Methods. Patients w ith SS whose disease began before age 16 were identified from a cohort study on SS. Previous patients with JSS published from 1952 to 1993 w ere found by literature review. Thirty-nine adult patients with primar y SS were selected as a control group. Results. Five patients with pri mary 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 sym ptom. An extraglandular manifestation (EGM) was the presenting feature in 9.3% of cases. Rheumatoid factor (RF) was positive in 71% and anti nuclear antibodies (ANA) in 67%. During the course of disease, at leas t one EGM was noted in 20 cases (51%). Leukopenia was the most frequen t 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 onse t was more frequent in children (62.5 vs 13%). The frequency of ANA an d articular manifestations were higher in adults (67 vs 92%, and 15.4 vs 38.5%, respectively). We reviewed 31 cases of secondary JSS, of whi ch the most common associated autoimmune disease was juvenile rheumato id arthritis (42%). JSS preceded by years the associated autoimmune di sease 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 ons et. Characteristics similar to those seen in adults are observed in ch ildren. Longterm followup is needed to assess outcome associated facto rs.