What is Systemic lupus erythematosus?

Lupus, like many other rheumatic diseases, is an autoimmune disease that ranges from mild to severe. The primary characteristics of the disease are recurrent injuries in the vessels throughout the body particularly in the skin, joints, blood, and kidney. SLE was first described in 1828. "Lupus ", comes from the Latin term for wolf, because the disease often produces a rash that extends across the bridge of the nose and upper cheekbones and resembling a wolf bite. The term erythematosus (from the Greek word for red) refers to the color of the rash, and the term systemic is used because the disease can affect organs and tissue throughout the body.

A malfunctioning immune system is the most likely cause of SLE. A healthy immune system produces antibodies, which are special proteins that help fight and destroy viruses, bacteria, and other foreign substances that invade the body. In lupus, the immune system produces antibodies that fight against the body's healthy cells and tissues. These antibodies, called autoantibodies cause inflammation in various parts of the body.

As is the case with rheumatoid arthritis, no one is really sure what causes this malfunction. It is likely that there is no one cause but rather a combination of genetic, environmental, chemical and possibly hormonal factors. The exact cause may differ from one person to another.

What are the symptoms?

Each person with lupus is different. No two will have the exact same symptoms with the same degree of severity. More common symptoms of lupus are extreme fatigue, painful or swollen joints, unexplained fever, and skin rashes. A characteristic skin rash may appear across the nose and cheeks, this is known as the butterfly rash. Other rashes occur elsewhere on the face and ears, upper arms, shoulders, chest, and hands. Other symptoms of lupus include chest pain, hair loss, sensitivity to the sun, anemia (a decrease in red blood cells), pale or purple fingers and toes from cold and stress, disturbances of thought and concentration, and personality changes.

How is it diagnosed?

According to the American College of Rheumatology, 4 of the following 11 symptoms indicate a diagnosis of SLE:

1. Characteristic rash across the cheek
2. Discoid lesion rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis
6. Inflammation of membranes in the lungs, the heart, or the abdomen
7. Evidence of kidney disease
8. Evidence of severe neurological disease
9. Blood disorders, including low red and white blood cell and platelet counts
10. Immunologic abnormalities
11. Positive antinuclear antibody

It is important to note that a negative ANA test makes a diagnosis of SLE unlikely but not impossible. Nor does a positive ANA definitely diagnosis SLE. Antinuclear antibodies may be strongly present in other autoimmune diseases like scleroderma, Sj?gren's syndrome and rheumatoid arthritis or weakly present in about 20% to 40% of healthy women.

How is it treated?

The treatment of lupus is tailored to each individual's symptoms.

Medications:
NSAIDS (Nonsteroidal anti-inflammatory drugs) are commonly used to treat the joint pain associated with SLE. Available over the counter or by prescription, they fight inflammation or swelling and relieve pain. NSAIDS are usually the first drug prescribed to a person newly diagnosed with rheumatoid arthritis. NSAIDS may relieve pain and swelling, but they do not slow the disease process.

DMARDS (disease modifying anti-rheumatic drugs) are also known as SAARDS (slow acting anti rheumatic drugs. As their name might suggest these medications take some time to be effective, up to several months. Most of them do not have any common properties other than their ability to slow down the progression of the disease.

Oral corticosteroids, such as prednisolone and prednisone (Deltasone, Orasone) usually work quickly to control inflammation. Due to the potential for serious side effects, long term use is usually discouraged. However if long term use is needed to maintain the control of inflammation, the lowest possible dose should be used.

Life style changes:
Simple changes can prevent or reduce the occurrence of certain symptoms. Avoiding overexposure to ultraviolet rays, wearing protective clothing and sunblocks, maintaining good dental hygiene, getting adequate rest of at least 8 hours and possibly a nap during the day, and eating a healthy diet are all important self-help measures.


What research is being done?

Identifying genes that play a part in the development of lupus is an active area of research. Researchers suspect a genetic defect in a cellular process called apoptosis, or "programmed cell death," in people with lupus. Apoptosis allows the body to safely get rid of damaged or potentially harmful cells. If there is a problem in the apoptosis process, harmful cells may stay around and do damage to the body's own tissues. Scientists are studying what role genes involved in apoptosis may play in human disease development.

Research to identify genes that predispose some people to the more serious complications of lupus, such as kidney disease, is producing significant findings. Researchers have identified a gene associated with an increased risk of lupus kidney disease in African Americans. They are also making progress in identifying other genes that play a role in lupus.

Using new information obtained about the disease process, scientists are using biologic agents to selectively block parts of the immune system. Development and testing of these new drugs, which are based on compounds that occur naturally in the body, is an exciting and promising new area of lupus research. The hope is that these treatments not only will be effective but also will have fewer side effects. Other treatment options currently being explored include reconstructing the immune system by bone marrow transplantation. In the future, gene therapy also may play an important role in lupus treatment.

Some of the related information found on Arthritis Insight:

For support visit our Chat Room and Message Boards.
For medication information see our Medication Index.
For information about tests and blood work go to Tests & Labs.
To find tips for getting through the day, stop by our Better Living section.
For more sites about Lupus check out our Web Links.

For more information:

SLE: by drdoc online 
Lupus Around the World 
The Santa Barbara Lupus and Rheumatic Diseases Group Home Page 
Lupus Foundation of America