CURRENT MORTALITY AND RISK FACTORS
FOR SURVIVAL IN CHILDREN WITH SYSTEMIC LUPUS ERYTHEMATOSUS IN JAPAN
Takei S,
Imanaka H, Naeno N, Mori H, Nerome Y, Hokinohara M, Miyata K
Department of
Pediatrics, Kagoshima University, Kagoshima, Japan
Objective: To investigate the current survifal
rate and risk factors for mortality in children with systemic lupus erythematosus
(SLE) in Japan.
Methods: Demographic, clinical, and laboratory
variables, obtained from a nationwide survey in 1995, was analyzed by
logrank test and Cox hazard regression model based on Kaplan-Meier
life-table analysis.
Results: A total of 335 lupus children, 52 boys
and 283 girls, observed mean 6.0 years were analyzed. The overall survival
rate was 98.3% at 5 years and 92.4% at 10 years. The logrank test
identified the following risk factors for mortality: central nervous system
(CNS) disease resulting in neuropsychiatric symptoms, convulsion and
paralysis; and family history of collagen vascular disease. Sex, age at
onset, and lupus nephritis-including Class IV nephritis at the initial
biopsy ¨C were not correlated with mortality. The Cox regression model
indicated that pericarditis, neuropsychiatric symptoms, family history of
collagen vascular diseases, convulsions, and paralysis were significant
risk factors for mortality by univariate analysis.
Conclusion:
Remarkable improvement
is observed in the mortality rate of lupus children in Japan. CNS
involvement, however, remains a risk factor for mortality, whereas
nephritis does not influence survival. Thus, management of treatment
complications and of chronic organ dysfunction has emerged as the major
focus in the care of children with SLE.