Objective. To investigate the prevalence of anti-alpha -fodrin antibody spe
cific 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 r
ecombinant 120 kDa alpha -fodrin fusion protein.
Results. All the 15 serum samples from patients with SS reacted with a reco
mbinant a-fodrin fusion protein in Western blot analysis. In contrast, reac
tivity was found in only 2 of the 16 patients with SLE, The clinical featur
es of the 15 patients with juvenile onset SS were very specific; only 4 pat
ients complained of dryness, while 6 had abnormal excretion ability. Saliva
ry gland enlargement was the most common clinical manifestation. Characteri
stic laboratory findings in juvenile onset SS included a higher prevalence
of antinuclear antibodies, anti-SSA/Ro antibodies, and rheumatoid factor, a
s well as increased erythrocyte sedimentation rate and hypergammaglobulinem
ia.
Conclusion, The pathogenesis of juvenile SS seems to be: the same as that o
f adult SS, although subjective symptoms of dryness are less frequent in ju
venile cases. This discrepancy may indicate that SS is a slowly progressive
disease with a long time span. The anti-alpha -fodrin antibody is likely t
o he a reliable diagnostic marker for juvenile SS.