Systemic Lupus Erythematosus

What Is Systemic Lupus Erythematosus?

Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown cause. The disease is characterized by inflammation and disordered function of potentially many different body parts. Antibodies formed against various cell parts are seen in this disease. These antibodies may take a direct part in the abnormalities seen by directly injuring cells and organs, or by participating in protein complexes, which can be deposited in organs to cause dysfunction. Sometimes these antibodies “sensitize” cells so that the host (patient) sees a “foreign” cell and begins an attack, essentially on herself/himself. Sometimes blood vessels can become involved in the autoimmune process, so that the organs these blood vessels supply can be damaged.

Who Develops SLE?

SLE is primarily a disease of young women. Most cases occur during child-bearing years, and in fact, SLE may be made worse by pregnancy. Men may also develop SLE, but in an incidence 5 times lower than women. SLE may occur in children, as well. There is even neonatal lupus occurring at birth (in children born to mothers with SLE or mothers with certain antibodies.) There is also most likely a hereditary predisposition to SLE, which we have seen by studying families and siblings with the disease. However, the exact gene involved (if there is one) is not known.

What Are The Manifestations Of SLE?

The manifestations of SLE are many and varied. As a matter of fact, the signs of lupus may be the same as signs of multiple other diseases, and, vice versa. This fact makes it imperative for the patient to have a complete history and physical by a trained physician, along with the appropriate and specific laboratory and radiologic testing performed, before the physician may arrive at a diagnosis of SLE.

A common sign of SLE is the malar rash, or, so-called, butterfly rash. This is usually in the distribution over the bridge of the nose and on the cheeks (like butterfly wings). But the same rash of SLE may occur on almost any part of the body. Usually these rashes are made worse by exposure to the sun or other UV light (and in some cases, even with exposure to fluorescent lights.)

Inflammatory arthritis may be seen in SLE. In the early stages, this joint swelling may be mistaken for rheumatoid arthritis, but as time passes, the characteristics of SLE arthritis may change. Joints may develop deformities, and there may even be nodule formation.

Inflammation in the connective tissue of the heart and lungs may cause significant problems in SLE. The lining of the lung may be involved and pleurisy results. Scarring may occur in the interstitium causing shortness of breath. In severe cases, the lungs may hemorrhage. Fluid may accumulate around the heart causing pericarditis. Abnormalities in the heart valves, blood vessels, muscle of the heart, or even in the conduction system may become involved.

The kidney is a prime target in severe SLE. Antibodies attack the kidney and immune complexes of antibodies and other inflammatory proteins may settle out in the tiny blood vessels of the kidney causing disease. This can be manifested by protein in the urine, and sometimes blood or “casts” of cells may be seen. High blood pressure may result. Loss of kidney function can progress to kidney death necessitating dialysis.

Other body parts involved may include the mucous membranes, blood vessels, brain, nerves, gastrointestinal system, muscles, etc. Gratefully, severe SLE involving multiple organs is rare. And, also, gratefully, most manifestations if detected promptly can be managed successfully.

Treatment Of SLE

The treatment of SLE is individualized to fit the patient’s needs. For example, if skin abnormalities are the only manifestation of SLE in a patient, then topical steroids may be indicated, without the use of oral medications. The arthritis of SLE may respond to nonsteroidal anti-inflammatory medications. The antimalarial medications are very helpful in controlling skin SLE and in helping the arthritis of SLE. Many times, however, the inflammatory manifestations of SLE can only be controlled through the use of systemic corticosteroids. Kidney disease especially requires high doses of steroids. In addition, cytotoxic medications may need to be given for severe, unrelenting disease. Novel therapies such as stem cell transplantation are being studied in SLE. Occasionally, combinations of therapies may be indicated. Besides treating the manifestations of SLE, the physician and patient must work together to minimize the side effects of medications used and to help maintain overall good health.

Authored by: Carolyn Smith, M.D.

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