Causes of death — The major cause of death in the first few years of illness is active disease (eg, CNS, renal, or cardiovascular disease) or infection due to immunosuppression, while late deaths are caused by the illness (eg, end-stage renal disease), by treatment complications (including infection and coronary disease), by non-Hodgkin lymphoma, and by lung cancer [141,143-147]. The frequency of the different causes of death can be illustrated by the following observations:
- The largest study included survival data and causes of death in a total of 9547 patients who were followed for an average of 8.1 years [147]. Standardized mortality rates (SMR) of SLE patients to expected rates for a age- and sex-adjusted population were noted for circulatory disease (SMR 1.7), especially heart disease (SMR 1.7); for non-Hodgkin lymphoma (SMR 2.8); for lung cancer (SMR 19.4); for infections (SMR 9.0), especially pneumonia (SMR 7.2); and for renal disease (SMR 4.3). Those at particularly high risk for mortality were younger, female, and black, with disease duration of less than one year.
- One study evaluated the causes of death in 408 patients with SLE followed over a mean period of 11 years; 144 (35 percent) died [148]. The major causes of death were active lupus (34 percent), infection (22 percent), cardiovascular disease (16 percent), and cancer (6 percent). Deaths that resulted directly from SLE and infection were common among younger patients; the risk of death directly due to SLE was highest in the first three years after diagnosis.
- Another prospective study followed 1000 patients for 10 years [135]. The most frequent causes of death were active SLE (26 percent), infection (25 percent), and thromboses (26 percent) [135].
- In a cohort of 4747 Swedish patients who were diagnosed with SLE between 1964 and 1995, the proportions of deaths due to cardiovascular events, SLE, and malignant disease were 42 percent, 21 percent, and 12 percent, respectively [149].
Serious infection is most often due to immunosuppressive therapy. (See "Secondary immune deficiency induced by drugs and biologics".) Patients at particular risk are those treated with both glucocorticoids and cyclophosphamide, especially if the white blood cell count is less than 3000/microL and/or if high-dose glucocorticoids are given [150,151]. Lymphopenia (<1000/microL) at presentation may be an independent risk factor [152]. (See "General toxicity of cyclophosphamide and chlorambucil in inflammatory diseases" and "Major side effects of systemic glucocorticoids".)
Premature coronary artery disease is being increasingly recognized as a cause of late mortality. (See "Coronary heart disease in systemic lupus erythematosus".)