Systemic lupus erythematosus (SLE)-related mortality has significantly declined in Japan during the past four decades. Decrease in number of deaths due to renal failure has considerably improved the prognosis of SLE. The adequate use of steroid therapy, the new advances in the second-line therapies including immunosuppressants, plasmapheresis and hemodialysis, and the appropriate control of infectious complications are the major reasons of the reduction of SLE-related mortality. The establishment of a nation-wide registration system of SLE in Japan will allow us to improve our strategy to control the disease.