This population-based study in Hawaii collected cases of systemic lupus erythematosus from medical facilities and a patient support group. A total of 454 cases was found and the prevalence was estimated at 41.8 per 100,000 population for 1989. The prevalence odds ratio for all non-Caucasians compared to Caucasians was 1.2 (0.9-1.5), for Japanese 1.3 (1.0-1.7), for Filipinos 1.5 (1.1-2.0), for Chinese 2.4 (1.7-3.4), and for Hawaiians 0.8 (0.6-1.1). Mortality rates were 3 times higher for non-Caucasians than for Caucasians in 1985 to 1989. Despite the observed differences, it remains unclear whether individuals with Asian or Pacific Islander ancestry are at higher risk for systemic lupus erythematosus. The variation in prevalence rates may be a result of differences in access to medical care and in survival.