Anti-alpha-fodrin antibodies in Sjögren's syndrome in children.
OBJECTIVE
To investigate the prevalence of anti-alpha-fodrin antibody specific for adult Sjögren'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 alpha-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.


CONCLUSIONS
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 be a reliable diagnostic marker for juvenile SS.
Maeno
N
N
Department of Pediatrics, Faculty of Medicine, Kagoshima University, Kagoshima City, Japan.
Takei
S
S
Imanaka
H
H
Oda
H
H
Yanagi
K
K
Hayashi
Y
Y
Miyata
K
K
D000293:Adolescent; D001323:Autoantibodies / Q000032:analysis*; D015153:Blotting, Western; D002352:Carrier Proteins / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000276:immunology / Q000503:physiopathology; D008297:Male; D008840:Microfilament Proteins / Q000276:immunology*; D012859:Sjogren's Syndrome / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D013049:Spectrin
D001323:Autoantibodies; D002352:Carrier Proteins; D008840:Microfilament Proteins; D013049:Spectrin