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 rarely 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 than Caucasians, Asian-Americans, Latinos, or Native-Americans in the U.S. 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 ten times more often than males.

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:

  • autoimmune diseases, such as juvenile diabetes, rheumatoid arthritis, and anemia
Lupus

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:

  • malar rash - a rash shaped like a butterfly that is usually found on the bridge of the nose and the cheeks.
  • discoid rash - a raised rash found on the head, arms, chest, or back.
  • fever
  • inflammation of the joints
  • sunlight sensitivity
  • hair loss
  • mouth ulcers
  • fluid around the lungs, heart, or other organs
  • kidney problems
  • low white blood cell or low platelet count
  • Raynaud's phenomenon - a condition in which the blood vessels of the fingers and toes go into spasm when triggered by factors such as cold, stress, or illness.
  • weight loss
  • nerve or brain dysfunction
  • anemia

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:

  • blood test - to detect for certain antibodies that are present in most people with lupus.
  • blood and urine tests - to assess kidney function.
  • complement test - to measure the level of complement, a group of proteins in the blood that help destroy foreign substances (low levels of complement in the blood are often associated with lupus).
  • x-rays - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.

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:

  • malar rash - a rash shaped like a butterfly that is usually found of the bridge of the nose and the cheeks.
  • discoid rash - a raised rash usually found on the head, arms, chest, or back.
  • sunlight sensitivity
  • mouth ulcers
  • inflammation of the joints
  • heart or lung involvement
  • kidney problems
  • seizures or other neurological problems
  • positive blood tests
  • changes in normal blood values

Treatment for lupus:

There is currently no cure for lupus, however, symptoms can be controlled with treatment, and children/teens can lead a normal life. Specific treatment for lupus will be determined by your child's physician based on:

  • your child's overall health and medical history
  • extent of the condition
  • your child's tolerance for specific medications, procedures, and therapies
  • expectation for the course of the disease
  • specific organs that are affected
  • your opinion or preference

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:

  • hydroxychloroquine
  • corticosteroids (to control inflammation)
  • immunosuppressive medication (to suppress the body's autoimmune system)
  • liberal use of sunscreen, decreased time outdoors between 10:00 a.m. and 4:00 p.m., and wearing hats and long sleeves when outdoors, as about one-third of persons with lupus have the tendency to develop a rash in the sun
  • rest, including at least eight to ten hours of sleep at night; naps and breaks during the day
  • stress reduction
  • well-balanced diet
  • immediate treatment of infections

Consult your child's physician regarding which vaccines or immunizations are appropriate for children with lupus.