Systemic lupus erythematosus has many guises, but the unifying feature is the presence of antibodies against double-stranded DNA in almost all patients. This review provides data that show that such autoantibodies cause the renal lesions of systemic lupus erythematosus, and it emphasizes the importance of histones, histone fragments, and other nuclear autoantigens.

(1) Early symptoms include fever and lesion of the skin and joints. Typical skin lesion shows the symmetrical butterfly erthema on both cheeks and nose. In severe cases, skin lesion spreads all over the body, mucous membranes of the mouth and vulva erode and ulcerate, and the hair falls out. In most cases, there appears irregular low fever and high fever in occasional cases. Arthralgia mostly involves the joint of the limbs and Raynaud phenomenon may occur in those cases who are exposed to cold. At the later stage, injury and even failure of multiple organs appear.

(2) Different symptoms are seen when various organs are involved at the later stage. The kidneys are first involved and injured severely. So nephritis and nephritic syndrome occur and ultimately uremia may appear. In addition, the cardiovascular system, the respiratory systern, the digestive system and the nervous system may get involved and corresponding symptoms are seen.

(3) Laboratory examination, anemia, decreased numbers of white blood cells, lymphocytic cells and platelets, and increased ESR; elevated serum gamma globulin, IgG, IgM, IgA and circulatory immunocomplex; decreased complement; positive ANA and anti-ds-DNA-antibody; proteinuria seen in the patients with the kidneys involved.

(4) Skin LBT is positive or specific change is found in renal tissue biopsy.