Systemic Lupus Erythematosus
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Systemic Lupus Erythematosus (SLE) is a chronic, usually life-long, potentially fatal autoimmune disease characterized by unpredictable exacerbations and remissions with protean clinical manifestations. In SLE there is a predilection for clinical involvement of the joints, skin, kidney, brain, serosa, lug, heart and gastrointestinal tract. Women and minorities are disproportionately affected and SLE is most common in women of child-bearing age although it has ben reported in both extremes of life (e.g. diagnosed in infants and in the tenth decade of life). The prevalence in the Unites States had been estimated as approximately 500,000 but a recent telephone survey commissioned by the Lupus Foundation of America suggested a prevalence of as many as 2,000,000. A recent study identified a prevalence of 500 per 100,000 (1:200) in women residing in the area surrounding Birmingham, Alabama.
The prognosis for patients with SLE has greatly improved over the last few decades with at least 80-90% of all patients surviving ten years. Thereafter life expectancy approximates that of age matched controls. This improvement reflects the general advancements in health care (i.e. dialysis, antibiotics, antihypertensives, newer immunosuppressives with more favorable efficacy to toxicity ration) but also the specialized care available for patients with SLE.
SLE is a complex disorder affecting a predominately young population and shares similarities with HIV infection as regards the propensity for multiple organ involvement, potentially life-threatening episodes, and need for sophisticated monitoring. Patients with SLE are appropriate for a Center of Excellence since medical care by experienced clinicians with access to state of the art diagnostic and therapeutic measures will result in improved outcomes and the most cost-effective utilization of resources. Expert care of patients with SLE will lead to fewer hospitalizations secondary to uncontrolled disease exacerbation, less severe renal disease with fewer patients experiencing end stage renal disease requiring chronic dialysis, fewer episodes of avascular necrosis requiring total joint replacement, and less severe osteoporosis and fractures. More judicious use of steroids and steroid sparing agents can also reduce the severity of atherosclerosis and resulting incidence of myocardial infarctions and cerebral vascular accidents which now complicate the natural history of SLE. Preventive