Sjögren's syndrome in childhood.
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.


CONCLUSIONS
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.
Anaya
J M
JM
Department of Medicine, University of Texas Health Science Center at San Antonio (UTHSCSA) 78284-7874, USA.
Ogawa
N
N
Talal
N
N
D000293:Adolescent; D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis; D001172:Arthritis, Rheumatoid / Q000150:complications; D001327:Autoimmune Diseases / Q000150:complications; D005260:Female; D015803:HLA-DR3 Antigen / Q000032:analysis; D006801:Humans; D007592:Joint Diseases / Q000209:etiology; D007970:Leukopenia / Q000209:etiology; D010309:Parotitis / Q000209:etiology; D012217:Rheumatoid Factor / Q000032:analysis; D012859:Sjogren's Syndrome / Q000150:complications* / Q000276:immunology / Q000503:physiopathology*
D000974:Antibodies, Antinuclear; D015803:HLA-DR3 Antigen; D012217:Rheumatoid Factor