As a pediatric rheumatologist, I diagnose and treat kids with chronic autoimmune diseases. "Chronic" means that it can last for a long time. "Autoimmune" means that there is a disorder of the immune system, which, instead of protecting the body from bacteria and viruses, attacks the patient’s own tissues. There are over 100 autoimmune diseases.
What is lupus?
There are different types of lupus: skin only, systemic (affecting multiple organs), drug induced.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect various organs of the body, especially the skin, joints, blood, kidneys and central nervous system. The name “SLE” dates back to the early 20th century. "Systemic" means that it affects many organs of the body. The word "lupus" is from the Latin word for "wolf" and it refers to the characteristic butterfly-like rash on the face of some lupus patients, which is similar to the white markings on a wolf’s face. "Erythematosus" in Greek means "red" and it refers to the redness of the rash.
How common is it?
SLE is seen worldwide in both children and adults. Childhood onset SLE (cSLE) represents 15 to 20 percent of all SLE patients, affecting about ten out of one million children per year. Onset of SLE is rare before five years of age. It is more common in children of African American, Hispanic, Asian, and Native American origin.
What causes lupus?
The exact causes of SLE are unknown. What is known is that SLE is an autoimmune disease, where the immune system loses its ability to tell the difference between a foreign intruder and a person’s own tissues and cells. The immune system produces autoantibodies that identify the person’s own normal cells as foreign, and eliminates them. The result is an autoimmune reaction, which causes inflammation that effects specific organs (joints, kidneys, skin, or any organ where you have blood vessels).
Inflammation means that the affected body parts become hot, red, swollen and sometimes tender. If the signs of inflammation are long lasting, as they can be in SLE, then damage to the tissues may occur and normal function is impaired.
Is lupus genetic or infectious? Can I prevent it?
SLE is a multifactorial disease meaning It can be triggered by a number of factors, including environmental factors (such as sun exposure), viral infections (such as Epstein-Barr virus, which is the virus that causes mononucleosis), hormonal imbalance at puberty, stress and certain medications. SLE is not a genetic disease and it cannot be transmitted directly from parents to children. There is no prenatal testing to detect lupus during pregnancy
What are the symptoms?
The most common first symptoms in children are nonspecific complaints such as fatigue and malaise. Intermittent or prolonged fevers, weight loss and loss of appetite could also be seen. Over a period of several weeks, months, or even years some new symptoms may appear, including:
- A variety of different skin rashes – the typical “butterfly” rash across the nose and cheeks occurs in one-third to one-half of affected children
- Photosensitivity (where exposure to sunlight triggers a rash)
- Ulcers on the inside of the nose or mouth
- Hair loss (alopecia)
- Raynaud’s sign (the hands turn red, white or blue when exposed to the cold)
- Swollen and stiff joints (arthritis)
- Muscle pain and/or weakness
- Easy bruising
- Chest pain
Kidney involvement is present in most children with SLE and is a major complication of the long-term outcome of this disease. Symptoms of SLE can vary widely between each patient so each child's complaints are different. All of the symptoms mentioned above can occur at any time during the course of the disease. The diagnosis of SLE is based on a combination of symptoms (such as pain), signs (such as fever) and test results. Other illnesses must also be ruled out.
Is the disease different from adult lupus?
Children with lupus may have similar manifestations as adults. However, childhood onset lupus is usually a more severe illness and has greater disease damage over time. Kids with lupus may develop kidney and or brain disease within the first 2-3 years of the diagnosis. It has a lifetime burden.
How is it diagnosed?
Clinical suspicion for lupus is the key to diagnosis. To make a formal diagnosis of SLE, the patient must have at least 4 out of 11 characteristics outlined by ACR (American College of Rheumatology). Laboratory tests can help diagnose SLE and decide which internal organs, if any, are involved. Many other tests are also available to look at the effects of SLE on different parts of the body. A biopsy (the removal of a small piece of tissue) of a kidney or skin is often performed when there is suspicion.
Can it be treated or cured?
At this time, there is no cure for SLE. SLE treatment can help control symptoms of SLE and help prevent complications of the disease, including permanent damage to the organs and tissues. When SLE is first diagnosed, it is usually very active and may require high doses of medications to control the disease and prevent organ damage. In many children, treatment brings lupus flares under control. The disease may go into remission and little or no treatment is needed.
Lupus is a chronic, multi-system disease of inflammation that can consist of flares and remissions. Expert care is key to controlling this unpredictable disease. Nationwide Children's Hospital's Division of Rheumatology recently started Lupus Clinic for a multidisciplinary approach to children with lupus.
For more information on Nationwide Children’s Hospital Rheumatology Services and Research, click here.