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Systemic Lupus Erythematosus (SLE, or Lupus)

What is lupus?

Systemic lupus erythematosus, also known as SLE, or simply lupus, is a disease that is characterized by periodic episodes of inflammation of and damage to the joints, tendons, other connective tissues, and organs, including the heart, lungs, blood vessels, brain, kidneys, and skin. The heart, lungs, kidneys, and brain are the organs most affected. Lupus affects each individual differently and the effects of the illness range from mild to severe. Lupus can potentially be fatal.

The majority of people who have lupus are young women (late teens to 30s). This may be due to the fact that estrogen (a female hormone) seems to be associated with SLE. Lupus affects more African Americans, Asian Americans, Latinos, and Native Americans than Caucasian Americans. Lupus in children occurs most often at the age of ten and older; lupus is rare in children younger than 5 years of age.

The disease is known to have periods of flare-ups and periods of remission (partial or complete lack of symptoms). Children with lupus can have a large degree of kidney involvement. The severity of the kidney involvement can alter the survival rate of patients with lupus. In some cases, kidney damage is so severe it leads to kidney failure.

What causes lupus?

Lupus is an autoimmune disorder, which means the body's immune system attacks its own healthy cells and tissues.

Lupus is considered to be a multifactorial condition. Multifactorial inheritance means that "many factors" are involved in causing a health problem. The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait or condition. Often one gender (either males or females) is affected more frequently than the other in multifactorial traits. Multifactorial traits do recur in families because they are partly caused by genes. Females are affected with lupus three to 10 times more often than males.

A group of genes on chromosome 6 codes for the HLA antigens which play a major role in susceptibility and resistance to disease. Specific HLA antigens influence the development of many common disorders, many that are autoimmune related and are inherited as multifactorial traits. When a person has the specific HLA antigen type associated with the disease, they may have a genetic susceptibility to have the condition and be more apt to develop it. The HLA antigen associated with lupus is called DR2 and DR3. It is important to understand that a person without these antigens may also develop lupus, so that HLA antigen testing is not diagnostic or accurate for prediction of the condition.

What is the immune system?

The purpose of the immune system is to keep infectious microorganisms, such as certain bacteria, viruses, and fungi, out of the body, and to destroy any infectious microorganisms that do invade the body. The immune system is made up of a complex and vital network of cells and organs that protect the body from infection.

When the immune system does not function properly, a number of diseases can occur. Allergies and hypersensitivity to certain substances are considered immune system disorders. In addition, the immune system plays a role in the rejection process of transplanted organs or tissue. Other examples of immune disorders include:

Anatomy of the immune system, child
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What are the symptoms of lupus?

Lupus symptoms are usually chronic and relapsing. The following are the most common symptoms of lupus. However, each child may experience symptoms differently. Symptoms may include:

Symptoms of lupus may resemble other medical conditions or problems. Always consult your child's physician for a diagnosis.

How is lupus diagnosed?

Lupus is difficult to diagnose because of the vagueness of the symptoms each child might have. There is no single test that can diagnose lupus. A diagnosis is usually confirmed based on your child's medical history, reported symptoms, and a physical examination that may include:

Further, the American College of Rheumatology created a set of criteria to assist physicians in making a diagnosis of lupus. The child must have four of the eleven specific criteria to be diagnosed with lupus. It is important to remember that having some of the following symptoms does not mean that your child has lupus. The criteria include:

Treatment for lupus:

There is no cure for lupus. Specific treatment for lupus will be determined by your child's physician based on:

If lupus symptoms are mild, treatment may not be necessary, other than possibly nonsteroidal anti-inflammatory medications (NSAIDs) for joint pain. Other treatment may include:

Children with lupus should not receive immunizations with live viruses, including chickenpox, MMR (measles, mumps, rubella), and oral polio vaccines. Consult your child's physician regarding all vaccines.

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