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Systemic lupus erythematosus (SLE or lupus) is an autoimmune disease associated with autoantibody production and immune complex deposition. The disease is heterogeneous in its clinical presentation, course, and prognosis. Despite many advances in understanding of the human immune system in recent decades, diagnostic and treatment approaches in SLE remain mostly the same. Patients with SLE have significantly increased morbidity and mortality. The risk of death in SLE patients is reported to be two to five times higher than the general population. Although the survival rate of patients with SLE has improved from a 4-year survival rate of 50% in 1950 to a 15-year survival rate of 85% in 2013, the mortality remains high compared to the general population and lupus nephritis (LN) outcomes have not changed in the past 30 years. Successful treatments, as measured by long-term remission, are limited, and at this time only one new medication—belimumab—has been approved for SLE in more than 60 years. Patients with SLE rarely achieve complete disease remissions with the currently available treatments.

Since SLE is a significantly heterogenous condition, the approach to management of patients should be tailored to each individual. In this chapter, we focus on current standard treatment approaches and discuss common associated issues.


Taking care of lupus patients is not merely about administering medications to treat their disease activity and manage their lupus-related symptoms but also about improving the quality of their lives through the prudent use of current therapies and preventing the damage that can result from both disease and treatment. Rheumatologists should pay specific attention to their patients’ lifestyles and encourage modifications that may be appropriate. Discussions about diet, exercise, smoking cessation, family planning, and sun protection are as important as the disease-specific treatments.

Most lupus patients see their rheumatologists more frequently than their primary care doctors. They have greater risk of cardiovascular disease compared to their age-matched group in general population. It becomes the rheumatologists’ responsibility to coordinate efforts to achieve better blood pressure and lipid metabolism in order to improve patients’ health and life expectancy.

Another complication of their disease and prolonged glucocorticoid treatment is osteoporosis. Rheumatologists taking care of lupus patients should assess their calcium and vitamin D intake regularly and screen them for osteoporosis. Majority of lupus patients have low serum levels of vitamin D, due at least in part to the avoidance of sun exposure and the use of sunscreens. Vitamin D levels should be monitored and repleted as needed.

Lupus patients are at increased risk of malignancy due to their proinflammatory state and immunosuppressive medication use. Routine screening for malignancy according to the guidelines for general population and preventive measures, such as human papillomavirus (HPV) vaccinations, are recommended.

Infection is still among the leading causes of death in lupus patients. Vaccination following the Centers for Disease Control ...

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