What is Systemic Lupus Erythematosus?

In general, Systemic Lupus Erythematosus (SLE) is a disease that affects girls and young women. Less commonly, SLE occurs in both older and young people and in boys. It often begins with fever, rash, joint pain and fatigue. Although these symptoms are worrisome, it is the ability of SLE affecting various internal organs that makes it a serious illness. Severe damage to the respiratory system, central nervous system, or to the kidneys can cause permanent disability or even death. It is important that children having the disease to be immediately identified and treated to prevent damage to internal organs when possible. Even without complaints of obvious symptoms, some children have found evidence of damage to internal organs.

Children and adults with SLE cannot turn off the immune response in their body; therefore patients have fever, aches everywhere, and often a rash. When these symptoms occur in the normal course of infection, the virus or bacterium are not the direct causes to the problems. Fever and pain and suffering are the result of the immune response to the infection. As the same time as different infections can damage different body parts, in children with SLE, uncontrolled immune response may produce different types of damages. Problems can occur anywhere, including the central nervous system, the cardiovascular system, respiratory system, muscles, kidneys, skin, joints, liver or intestines.

Normally, when areas are affected by SLE, antibodies, complement and inflammatory cells are present, and the tissue was damaged by the subsequent local inflammation. We begin to understand more clearly why the immune system fail to stop in children suffering SLE, but do not understand why some children develop a range of issues and problems while other children develop completely different problems. In animal models, genetics seems to be an important factor in determining which problems develop.

One of the most difficult issues for families of children with SLE is sun exposure. Everyone wants to play outside, go to the beach, sunbathe, and participate in normal outdoor activities. However, it is well established that exposure to ultraviolet light damages cells and causes cell death. "Programmed" cell death called apoptosis. This process enables damaged cells to self-destruct in an orderly manner and to be cleaned and removed from the body with minimal difficulty. However, this process is defective in the cells of patients with SLE and the damaged cells remain as a stimulant for the immune system for an extended period. This is thought to promote the formation of not only Antinuclear Antibody (ANA) but also other antibodies directed against important cellular elements that drive SLE as well. Consequently, significant exposure to sunlight can cause a severe exacerbation of the underlying SLE. It is essential that children with SLE using adequate sun block and avoid sun exposure. A single trip to the beach can cause life-threatening complications.

Systemic Lupus Erythematosus (SLE) mainly affects adolescent girls, but can affect both boys and girls of all ages. Although the typical butterfly rash on the face is regarded as characteristic of the disease, it was found in only one third of children during their first contact with a doctor's office. Given that many doctors do not believe it is SLE unless they seen the eruption of the rashes, many children have symptoms of SLE for months before having correct diagnosis. The key to rapid diagnosis of SLE are for the primary care physicians to consider this diagnosis whenever children being evaluated look chronically ill or having unexplained chronic damage to their organs (by example, kidney).

The most common symptom of SLE is fever. The second most common symptom is a chronic discomfort (malaise). The third most common manifestation is arthritis of small joints of hands and feet, often described as "hurting all over".

Despite chronic failure to thrive is the most common presentation, the SLE can also start in many other ways. Chronic inflammation of the hands and feet, bruising (with or without thrombocytopenia), joint pain, hematuria or proteinuria, photosensitivity, seizures, personality changes and depression, and poor school performance may be initial findings in children with SLE. However, it is important to remember that these are nonspecific symptoms that can occur in many different diseases.

The key to proper treatment of Systemic Lupus Erythematosus (SLE) is balancing the risks of disease with the risks of treatment. SLE is very diverse, and many children have only mild illness that can be easily treated with low doses of corticosteroids. Other children have severe, potentially fatal conditions that require aggressive treatment. There is no absolute standard to tell whether a child needs to be treated with immunosuppressive drugs. However, it is clear that prolonged therapy, even with moderate doses of corticosteroids, would cause significant side effects. Immunosuppressive drugs often seem to frighten parents and doctors who do not work with them all the time. While immunosuppressive drugs may be associated with serious side effects, these side effects are rare when it is administered by medical experts.

There is no perfect answer for children with SLE over time. Some doctors are reluctant to use cyclophosphamide, fearing the implications of the involvement of central nervous system, lung, or mild renal insufficiency. Cyclophosphamide does not help every child, every time. They can have possible side effects, but they are rare when it is administered by medical experts. Corticosteroids and azathioprine appears to be safer in the short term, but the results may not be that good in the long term.

For children with severe internal organs involvement, especially renal disease, most doctors agree that cyclophosphamide is the best answer. In carefully controlled studies, it is shown that cyclophosphamide prevents continued scarring of the kidney, while prednisone does not. No one has studied azathioprine in this regard. The academic answer is "We know that cyclophosphamide works. Why should we subject patients to a different therapy that might not work?"

Many studies combining newer drugs such as rituximab, abatacept and mycophenolate mofetil in various combinations, with and without cyclophosphamide, are now in course. The goal is to find a combination that immediately puts the disease into remission and keep it with the least risk of drug toxicity or recurrent SLE. No one has the best answer. Fortunately, there are relatively few children who fail standard therapy.