[The clinical manifestations of Sjögren's syndrome in children].
Sjögren's syndrome (SS) is thought to be uncommon in children. We studied the clinical manifestations and laboratory findings of 12 pediatric patients with SS, all of children did not have sicca symptoms but have lymphocytic infiltration of salivary glands, abnormal sialograms or abnormal results of scintigraphy compatible with typical SS. Seven cases had primary SS and five were secondary SS and had other autoimmune disorders (three cases with systemic lupus erythematosus, one case with dermatomyositis, and the other with mixed connective tissue disease). All patients were female. The mean age at onset of symptoms, including other autoimmune manifestations, was 12.2 years (range 9-15 years). The initial symptoms were some systemic manifestations (fever, exanthema, arthralgia, etc.) and various autoimmune phenomena (butterfly rash, Raynaud's phenomenon, proteinuria, weakness of muscles, etc.). On the other hand, no patients complained sicca symptoms. Laboratory studies in our patients revealed elevated levels of IgG (92%), antinuclear antibody (92%), rheumatoid factor (58%), anti-SS-A antibody (75%). These findings were similar to those found in adult patients with sicca symptoms previously reported in literature. From these studies, we suggest that lip biopsy, sialography and/or salivary gland's scintigraphy should be carried out in patients who had abnormal laboratory findings as mentioned above, irrespective of absence of sicca symptoms, in order to diagnose SS at early period.
Tomiita
M
M
Department of Pediatrics, School of Medicine, Chiba University.
Kohno
Y
Y
Honma
K
K
Aoyagi
M
M
Katsuki
T
T
Hoshioka
A
A
Saito
K
K
Shimojo
N
N
Kuroda
K
K
Tanabe
E
E
D000293:Adolescent; D000974:Antibodies, Antinuclear / Q000378:metabolism; D001706:Biopsy; D002648:Child; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D008046:Lip / Q000473:pathology; D011877:Radionuclide Imaging; D012217:Rheumatoid Factor / Q000378:metabolism; D012469:Salivary Glands / Q000000981:diagnostic imaging; D012796:Sialography; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000276:immunology
D000974:Antibodies, Antinuclear; D007074:Immunoglobulin G; D012217:Rheumatoid Factor