Twenty-six of 488 (5%) patients with systemic lupus erythematosus followed in a private practice developed clinically evident, symptomatic avascular necrosis. Avascular necrosis was significantly associated with hypertension, pleural effusions, cerebritis, nephritis, anemia, and earlier age of disease onset. No association with anticardiolipin antibodies or thromboembolic disease was observed. Avascular necrosis is usually observed in active, severe lupus.