Anti-α-fodrin antibodies in Sjogren's syndrome in children

Objective. To investigate the prevalence of anti-alpha -fodrin antibody specific for adult Sjogren's syndrome (SS) in patients with juvenile onset SS, Methods. Serum anti-alpha -fodrin antibody was examined in 15 patients with juvenile SS (11 cases of primary SS and 4 secondary SS) and in 16 children with systemic lupus erythematosus (SLE) by Western blot analysis using a recombinant 120 kDa alpha -fodrin fusion protein. Results. All the 15 serum samples from patients with SS reacted with a recombinant a-fodrin fusion protein in Western blot analysis. In contrast, reactivity was found in only 2 of the 16 patients with SLE, The clinical features of the 15 patients with juvenile onset SS were very specific; only 4 patients complained of dryness, while 6 had abnormal excretion ability. Salivary gland enlargement was the most common clinical manifestation. Characteristic laboratory findings in juvenile onset SS included a higher prevalence of antinuclear antibodies, anti-SSA/Ro antibodies, and rheumatoid factor, as well as increased erythrocyte sedimentation rate and hypergammaglobulinemia. Conclusion, The pathogenesis of juvenile SS seems to be: the same as that of adult SS, although subjective symptoms of dryness are less frequent in juvenile cases. This discrepancy may indicate that SS is a slowly progressive disease with a long time span. The anti-alpha -fodrin antibody is likely to he a reliable diagnostic marker for juvenile SS.