Prior to the availability of corticosteroids and immunosuppressive therapy, which became widely accessible after 1948, half of persons with lupus died within two years, and half survived. This generally demarcated organ- versus non-organ-threatening disease. Table 13.1 shows the gradual improvement in outcome over the past 60 years. Currently, most patients with systemic lupus erythematosus survive at least 20 years,1 although their quality of life is not always optimal. Historically, 40% of deaths in lupus patients with serious disease were from inflammation and occurred within two years of diagnosis. Approximately 10% of deaths took place over the following 10 years. The remaining 40% of lupus patients died 12 to 25 years after diagnosis, mostly from infections and complications of chronic steroid therapy and immunosuppression. This “bimodal” curve has been altered in the past 10 years.2 Death due to lupus during the first two years is becoming less common; individuals with serious SLE still have a 10- to 30-year shortened life expectancy due to complications of therapy.3 Figure 13.1 summarizes the principal causes of death in SLE.5, 6
Patients with drug-induced lupus, chronic cutaneous lupus, and non-organ-threatening SLE without antiphospholipid antibodies have a normal survival rate. The outcome of lupus patients depends on a variety of other factors (see Fig. 13.2): race; ethnicity; geography; access to health care; adherence to medication; gender; clinical and laboratory variables; treatment variables; presence of antiphospholipid syndrome; and attention to comorbidities such as accelerated atherogenesis, smoking cessation, weight reduction, patient education, and osteoporosis. Approximately 5% of persons with SLE experience spontaneous remission without treatment.
Over the past decade, a surprising number of approaches and interventions have been shown to improve the quality of life and outcome of patients with SLE without prescribing lupus medications.4
Table 13.1 Prognosis of lupus erythematosus
Figure 13.1 Early and late complications of systemic lupus erythematosus (SLE). Source: Urowitz MB, Bookman AA, Koehler BE, Gordon DA, Smythe HA, Ogryzlo MA. The bimodal mortality pattern of systemic lupus erythematosus. Am J Med. 1976;60(2):221–225. Reprinted with permission. Elsevier, ©1976.
Figure 13.2 Most common causes of death in SLE.
References
1. Abu-Shakra M, Urowitz MB, Gladman DD, et al. Mortality studies in systemic lupus erythematosus. Results from a single centre, 1. Causes of death. J Rheumatol. 1995;22:1259–1264.
2. Urowitz MB, Bookman AA, Koehler BE, et al. The bimodal mortality pattern of systemic lupus erythematosus. Am J Med. 1976; 60:221–225.
3. Ward MM. Hospital experience and mortality in patients with systemic lupus erythematosus. Arthritis Rheum. 1999;42:891–898.
4. Wallace DJ. Viewpoint: improving the prognosis of SLE without prescribing lupus drugs and the primary care paradox. Lupus. 2008;17:91–92.
5. Bernatsky S, Boivin J, Manzi S, et al. Mortality in systemic lupus erythematosus. Arthritis Rheum. 2006;54:2550–2557.
6. Bernatsky S, Levy D, Ramsey-Goldman R, Gordon C, Rahman A, Clarke AE. Mortality in SLE. In: DJ Wallace and BH Hahn, eds. Dubois’ Lupus Erythematosus and Related Syndromes, 8th ed. Philadelphia, PA: Elsevier; 2013:666–675.