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.