Objective: To describe the clinical characteristics of juvenile Sjögren'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. Previous 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. Long-term followup is needed to assess outcome associated factors.