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Systemic Lupus Erythematosus Summary

H. Michael Belmont, M.D.
Medical Director, Hospital for Joint Diseases
New York University Medical Center

Systemic Lupus Erythematosus (SLE), popularly known as Lupus, is an uncommon although not rare systemic rheumatic disease which is 9 to 10 times more frequent in women than men. Although lupus may occur at any age and in both sexes it is chiefly a disorder of women of child bearing age (70% or 7 out of every 10 with the disease are women between the age of 15 and 50). lupus is four times more common in African Americans than Caucasians and also affects Asians and Hispanics with an increased prevalence. It is estimated between one to one and a half million Americans suffer with this condition.

SLE is a classic autoimmune disease. The immune system is intended as a defense against invading infection (i.e. viruses, bacteria, and parasites) but in lupus this system goes awry and results in the formation of molecules such as autoantibodies, immune complexes, and complement which may attack organs of the body. Although lupus is a chronic, potentially life-long condition it is characterized by episodic activity. In other words, patients unpredictably experience disease flares followed by periods of disease inactivity. Additionally, during a flare, lupus may effect the skin, joints, kidney, brain, lung, heart and gastrointestinal tract although it is unlikely that in any patient all these systems would be involved. In fact, each lupus patient is unique and the severity, frequency, and extent of injury varies form patient to patient.

Flares result either from increased inflammation of the blood vessels within organs of the body or, in other instances, from the formation of blood clots within these vessels. Distinguishing between these two types of injuries is important. One is treated with anti-inflammatory medication, such as NSAIDs (nonsteroidal anti-inflammatory drugs), antimalarials (hydroxychloro-quine/plaquenil), steroids (prednisone, medrol), or in the severest situations chemotherapy (azathioprine/imuran, methotrexate, cyclophosphamide/cytoxan) while the other with medicines intended to prevent clotting, such as aspirin, ticlid, plavix, heparin, lovenox, or coumadin.

Approximately half of all SLE sufferers experience kidney inflammation. In 20%, despite appropriate treatment, kidney damage develops and progresses to end stage renal disease (ESRD). ESRD patients require dialysis, a blood purifying process which substitutes for damaged kidneys. Care by a Nephrologist (kidney specialis), kidney biopsy, and aggressive approaches with drug treatments such as steroids, intravenous solumedrol, intravenous cyclophosphamide, cyclosporine, or mycophenolate mophetil by be necessary in particularly difficult cases.

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While this overview is intended for those seeking technical understanding, a more general description of the disease can be found at "An Introduction to Lupus". Dr. Belmont has also posted a review of "Lupus Nephritis: Treatment Issues".

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