Long-term complications of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is still a disease with significant mortality. Although 5 yr after diagnosis 92% of patients are alive, the prognosis falls to 82% survival at 10 yr, 76% at 15 yr and only 68% at 20 yr in Toronto w1x. There has been improvement in survival, with the standardized mortality ratio in patients recruited to the Toronto cohort in 1970–1977 being 10.1 (95% CI 6.5–15.0), compared with 3.3 (95% CI 1.8–5.7) for those recruited between 1986 and 1994 w2x. Data from other centres in the USA and Europe has been similar. Studies published around 1980 found that about 80% of patients survived 5 yr and about 60% of patients survived 10 yr. More recent studies have shown that 5-yr survival is now nearer 90–95% and that 70–85% of patients survive 10 yr w3x. In most studies, patients with renal involvement have had a poorer prognosis than those without renal disease. Nevertheless, survival has shown improvement in those with renal disease presenting to a UK centre between 1976 and 1986 (81% 10-yr survival), compared with those presenting between 1963 and 1975 (56% 10-yr survival) w4x. The commonest cause of death has been infection, both in early and late deaths w1, 3x. Active SLE contributes to about a third of early deaths but less commonly to late deaths. However, deaths related to acute and chronic vascular disease including sudden death are more common in those dying more than 5 yr after diagnosis. However, there is more to prognosis than just death. There is considerable morbidity associated with more prolonged survival after the diagnosis of SLE. Most physicians caring for lupus patients will be familiar with patients in whom active disease has resolved but the patients have suffered from symptoms related to the accumulation of chronic damage w5x. Both active disease and damage can be associated with impaired quality of life and reduced functional ability, although other factors such as the psycho-social background of the patient will affect a patient’s perception of their disease as well w6x. Having improved therapy for active lupus disease, the challenge is now to understand and prevent the long-term complications of this disease, whether they are due to effects of the disease itself, the therapies used, or co-morbid disease (perhaps with associated underlying disease mechanisms or linked genetic predisposition).

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