Mortality Studies

Survival of patients with LE has increased significantly in the past 40 years. Studies performed in 1955 (Merrell and Shuldman 1955) showed a survival rate lower than 50% at 5 years, whereas recent studies (Abu-Shakra et al. 1995a, Gripenberg and Helve 1991, Pistinier et al. 1991, Seleznick and Fries 1991) indicate that approximately 93% of patients with LE survive more than 5 years, and 85% survive more than 10 years. In the Euro-Lupus project (Cervera et al. 2003), 92% survival was found after 10 years from the time of entry into the study, slightly higher than that found in the American studies. This is probably because of a more homogeneous care system in Europe and better management of patients with LE in the present decade (earlier diagnosis, more appropriately used anti-LE therapies, and advances in medical therapy in general).

The improved survival of patients with LE has been associated with an alteration in the patterns of mortality. In 1976, Urowitz et al. (Urowitz et al. 1976) described a bimodal pattern of mortality, emphasizing inflammatory activity as the principal cause of death in patients with a recent diagnosis of SLE, whereas cardiovascular events were the most important cause of death in those with long evolution. Recently, epidemiologic studies observed that although one third of the deaths can be attributed to SLE activity, the complications of the therapy and other noninflammatory manifestations are important causes of death in these patients. This is the case with antiphospholipid antibody-related thrombot-ic events ("antiphospholipid syndrome"), which were responsible for 26.7% of the deaths in the Euro-Lupus cohort.

Prognosis studies and investigation of variables affecting mortality in LE have identified a wide range of significant factors (Cervera et al. 2003, Fries et al. 1974, Ginzler et al. 1982, Pistiner et al. 1991, Reveille et al. 1990, Seleznick and Fries 1991, Studenski et al. 1987, Wallace et al. 1981). In the Euro-Lupus project, only nephropa-thy at the beginning of the study was found to have prognostic significance for lower survival probability. However, 88% of patients with nephropathy at the beginning of the study survived after a 10-year follow-up period. Other studies performed in the United States (Reveille et al. 1990) observed that black patients (who more frequently have renal disease) and those with worse socioeconomic conditions or a lower cultural level have the most aggressive course and greater mortality.

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