What is lupus?
Systemic lupus erythematosus, also known as SLE, or simply lupus, is a disease characterized by inflammation of — and damage to — the organs, skin and joints. The kidneys, heart, lungs and brain are the organs most commonly affected.
Lupus affects each child and adult differently. The effects of the illness can range from mild to severe, and lupus can also be fatal.
The majority of people who have lupus are young women between their late teens and 45 years of age. This may be due to the fact that estrogen (a female hormone) seems to be associated with lupus. Lupus affects more African-Americans, Asian-Americans, Latinos and American-Indians than Caucasian-Americans.
In children, Lupus is most common in those 15 or older. According to the Arthritis Foundation, about 25,000 children and adolescents have lupus or a related disorder.
The disease is known to have periods of flare-ups and remissions (partial or complete lack of symptoms).
Many children with lupus also have kidney problems. The severity of the kidney problems can worsen the survival rate of patients with lupus. In some cases, a child’s kidney damage is so severe that it leads to kidney failure and the possible need for a kidney transplant.
Lupus is an autoimmune disorder. In these disorders, the body's immune system attacks its own healthy cells and tissues.
Many factors are known to cause lupus. The factors are usually both genetic and environmental, and often affect one gender more than the other. Lupus can reoccur in families because it is at least partly caused by genes.
Signs and symptoms
Lupus symptoms are usually chronic, but they can become more and less severe during certain periods of a child’s life. Lupus affects each child differently but the following are the most common symptoms:
- 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
- Joint stiffness, pain and swelling
- Sunlight sensitivity
- Hair loss
- Mouth ulcers (sores)
- 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
- Low red blood cell counts
- Decreased appetite
- Low energy
- Swollen lymph nodes
Symptoms of lupus may resemble other medical conditions or problems. If you are concerned, consult with your child’s doctor.
It is important to remember that having some of the above symptoms does not mean that your child has lupus. Always consult with a physician for a complete diagnosis and treatment plan.
Testing and diagnosis
Lupus is difficult to diagnose because of the broad range and severity of symptoms each person might have. There is no single test to diagnose lupus. Instead, clinicians usually make a diagnosis based on your child’s medical history, reported symptoms, a physical examination, and a variety of diagnostic and imaging tests.
Tests may include:
- Blood tests to detect certain antibodies that are present in almost all 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 to produce images of internal tissues, bones and organs.
- Erythrocyte sedimentation rate (also called ESR or sed rate) to measure how quickly red blood cells fall to the bottom of a test tube. When swelling and inflammation are present, the blood's proteins clump together and become heavier than normal. Thus, when measured, they fall and settle faster at the bottom of the test tube. Generally, the faster the blood cells fall, the more severe the inflammation.
- C-reactive protein (CRP) test is used to detect inflammation in the body. Although ESR and CRP reflect similar degrees of inflammation, sometimes one will be elevated when the other is not. This test may be repeated to test your child’s response to medication.
There is no cure for lupus, but treatment can relieve some of the symptoms of the disorder.
Treatment will be determined by your child’s doctor based on:
- Your child’s age, overall health and medical history
- Extent of the condition
- Your child’s tolerance for specific medications, procedures and therapies
- Your 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 drugs (NSAIDs) for joint pain.
Other treatment may include:
- Hydroxychloroquine medications to ease symptoms.
- Corticosteroids to control inflammation.
- Immunosuppressive medication to suppress the body's autoimmune system.
- Monoclonal antibodies, such as belimumab and rituximab, may be used for select patients, depending on disease activity and results of certain blood tests. Patients respond differently to these treatments and are carefully selected by their doctor.
- Liberal use of sunscreen, decreased time outdoors between 10 a.m. and 4 p.m., and wearing hats and long sleeves when outdoors. The sun can cause the disease to get worse.
- Rest, including at least 8-10 hours of sleep at night.
- Stress reduction.
- Well-balanced diet.
- Immediate treatment of infections.
Children with lupus should not receive immunizations with live viruses, including chickenpox, MMR (measles, mumps, rubella), and oral polio vaccines. Consult your child's doctor regarding all vaccines.
Children with lupus require frequent monitoring by a rheumatologist to make sure the disease is under control and medications are not having side effects. Depending on what organs lupus is effecting, children may require care from specialists who take care of the kidneys (nephrologist), lungs (pulmonologist), skin (dermatologist), brain (neurologist) or heart (cardiologist).
There is no cure for lupus, but, in most cases, symptoms can be controlled through lifestyle changes and medications. Because having a chronic disease such as lupus is very stressful, children with lupus often benefit from seeing a mental health counselor such as a psychologist or licensed clinical social worker.
Reviewed by Jay Mehta, MD, MS