To elucidate the clinical characteristics of childhood onset systemic lupus erythematosus (SLE), we carried out a retrospective reivew on medical records of fourteen patients who were diagnosed as SLE on the base of "the 1982 revised criteria for
the
SLE"(by american Rheumatism Association) at the Department of pediatrics, Hanyang University Hospital from January 1980 through August 1992.
@ES The results were summerized as follows:
@EN 1) Eleven girls and three boys were recruited in our study, The eight of them were early teens at the time of SLE diagnosis.
2) The frequencies of their clinical manifestations on admission were persistent facial rashes, edema, hepatosplenomegaly, renal abnormalities were proteinuria and hematuria.
3) Immunologic abnormalities were noted as follows:
Positive ANA 100%. positive LE cell 64%, elevated anti DS-DNA antibody 64%, and decreased only C3 level 28%, decreased both C3 & C4 64% of the patients.
4) Serum anti-DS DNA(>10units) titer was increased in clinical exacerbated three patients and decreased in four resolved.
5) The high ANA titers( >1;80)obtained in eleven and/or homogenous pattern shown in seven strongly suggested SLE.
6) Renal biopsies of the four patients revealed lupus nephritis and three of the four patients had lupus band in their biopsied skin.
7) Three of 14 patients were recoverd spontaneously with supportive care. Other 11 patients were treated: 7 with prednisolone only, 4 combined prednisolone and other agents. After treatment five were clinically improved. But 4 patients were
frequently
exacerbated over 2 years, and 2 patients were died of their complications.
In conclusion, serum anti-DS DNA level and ANA may be useful for the screening of SLE and for predicting clinical course and prognosis of SLE.
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