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Diseases & Conditions

Systemic Lupus Erythematosus in Children

Systemic lupus erythematosus (SLE) is an autoimmune disease, which means that the immune system mistakenly attacks the body’s own tissues. This causes inflammation (swelling) in the skin, kidneys, lungs, nervous system, and other organs.

Systemic lupus erythematosus is very rarely seen in people before the age of 10. It is more common in females and in some ethnic groups, such as African-American, Hispanic, South and Southeast Asian, and Native American.

Systemic lupus erythematosus affects children in the same ways that it affects adults. The main difference is that the care of children is different from that of adults because treatment can have a great impact on a child’s physical and psychological growth and development.

What are the symptoms of systemic lupus erythematosus in children?

Parents of children who have systemic lupus erythematosus often seek out a doctor due to the following symptoms:

  • Fever
  • Fatigue
  • Weight loss
  • Hair loss
  • Stomach pain
  • Rashes on the face or upper body
  • Headaches
  • Easy bruising
  • Painful joints
  • Seizures or psychosis
  • Decline in school performance
  • Symptoms of anxiety or depression

What causes systemic lupus erythematosus?

The exact causes of systemic lupus erythematosus are unknown. In general, patients who have lupus are born with genetic factors that predispose them to developing the disease. However, not all patients with these genetic findings develop lupus – some environmental trigger kicks off a response in the immune system to cause lupus. These possible triggers include:

  • Epstein-Barr virus
  • Sun exposure
  • Drug reaction
  • Exposure to cigarette smoke
  • Hormones during puberty

How is systemic lupus erythematosus diagnosed?

Systemic lupus erythematosus is diagnosed if four or more of the following symptoms occur, either over a period of time or at the same time, with no other explanation for their appearance:

  • Red rash on the cheeks and bridge of the nose (appears in about one-third of cases)
  • Disc-shaped rash with raised patches, with possible scarring from previous outbreaks
  • Skin rash due to sun exposure
  • Sores in the mouth or nose
  • Arthritis in two or more of the small joints
  • Fluid surrounding the heart or lungs
  • Kidney problems
  • Seizures or psychosis
  • Low red blood cell count (anemia), platelets, lymph or white blood cells
  • Abnormal tests for any of a number of immune system antibodies
  • High laboratory readings for anti-nuclear antibodies (ANA), a type of immune system cell that attacks the nuclei of cells

The doctor will take other factors into account, such as family history, age, and how long the patient has had symptoms, before making a diagnosis.

How is systemic lupus erythematosus treated?

There is no cure for systemic lupus erythematosus, but proper treatment can help greatly. The main goal of treatment is to ease symptoms and to prevent the immune system from attacking vital organs before permanent damage occurs. When treating children, this requires the attention of a team of specialists, including a pediatric rheumatologist, nurses, counselors and physical therapists who can provide necessary treatment to ensure the child’s physical and psychological health.

Counseling may be necessary to help the child or teen deal with the fact that he or she has the disease, and to stay motivated to keep doctors’ appointments, get regular blood tests, and take medications.

Medications will vary, depending on how severe the patient’s symptoms are. Medications include:

  • Corticosteroids (prednisone) to control inflammation;
  • Hydroxychloroquine (Plaquenil), an anti-malaria drug used to control disease flareups;
  • Immunosupressants such as azathioprine (Imuran), mycophenolate mofetil (Cellcept), methotrexate, cyclophosphamide (Cytoxan), and rituximab (Rituxan);
  • Pain relievers such as ibuprofen or naproxen;
  • Calcium and vitamin D supplements to prevent osteoporosis (a possible side effect of long-term use of corticosteroids)

What happens after treatment for systemic lupus erythematosus?

Prednisone is one of the most effective treatments for controlling inflammation in lupus. There may be serious side effects, however, such as a suppressed immune system, weight gain, stretch marks, high blood pressure, osteoporosis, depression, diabetes, glaucoma, and cataracts. These conditions can have a big impact on the child’s appearance, psychological well-being, and normal growth and development. Therefore, it is important for the patient to see his or her doctor on a regular basis to keep track of these side effects.

Hydroxychloroquine may, in rare cases, cause permanent damage to the retina. All children receiving the drug should have regular checkups with an eye doctor.

With proper treatment, children with systemic lupus erythematosus can continue to attend school, go out with friends, exercise, and take part in family activities.

What is the long-term outlook for children who have systemic lupus erythematosus?

Children who receive proper treatment for systemic lupus erythematosus generally have very good outcomes. The survival rate is nearly 100% at five years following treatment, and 85% at 10 years. Children with systemic lupus erythematosus may have a greater risk for early atherosclerosis (clogging of the arteries) and heart disease when they become young adults.

Other issues that may affect treatment include:

  • Failure to take medicines or follow the treatment
  • Problems with the nervous system
  • Other infections
  • Kidney disease
  • Difficulty getting proper care

Can systemic lupus erythematosus be prevented?

Since the causes of systemic lupus erythematosus are not fully understood, it is not known how to prevent it. Flare-ups of the condition may be reduced by:

  • avoiding sun exposure;
  • wearing sunscreen and protective clothing when going outdoors;
  • getting enough sleep; and
  • making sure to take the medications that have been prescribed.
References

National Institute of Arthritis and Musculoskeletal and Skin Diseases. Handout on Health: Systemic Lupus Erythematosus. www.niams.nih.gov. Accessed June 25, 2011.

Centers for Disease Control and Prevention. Systemic lupus erythematosus (systemic lupus erythematosus or lupus). www.cdc.gov. Accessed June 25, 2011.

Systemic lupus erythematosus in children. UptoDate. www.uptodate.com. Accessed June 29, 2011.

Lupus Foundation of America, Inc. Learning About Lupus. www.lupus.org. Accessed June 25, 2011.

American College of Rheumatology. Systemic lupus erythematosus in Children and Teens. www.rheumatology.org. Accessed June 25, 2011.

© Copyright 1995-2011 The Cleveland Clinic Foundation. All rights reserved.

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 6/30/2011…#14796