Systemic Lupus Erythematosus (SLE) in Children
What is lupus (systemic lupus erythematosus)?
Lupus (systemic lupus erythematosus) is a life-long autoimmune disease that causes joint pain, fever, rashes and organ damage. When you have an autoimmune disease, your immune system — which is supposed to protect you against threats like infections — attacks the body. This can lead to a wide variety of symptoms and even damage to organs like the heart, lungs, brain, kidneys and skin. Over time, this damage can result in serious complications.
In general, lupus affects children in the same way that it affects adults; however, lupus can be more severe, involve more organs and have more abnormal lab test results in children than adults. It is important to manage a child’s lupus symptoms over time to provide the best quality of life.
Does lupus (systemic lupus erythematosus) happen in children?
Systemic lupus erythematosus can happen in children. In most cases, lupus starts during a child’s teenage years (the average age is 12). It is rarely seen in children before the age of 5. Neonatal lupus can occur in a baby but this has no relationship to systemic lupus erythematosus. This is not very common.
Neonatal lupus is a disease in which the baby receives certain antibodies from the mother called Ro/SSA and or La/SSB, which are typically seen in Sjogren’s syndrome. The baby will usually have a rash, but they can also have heart problems, liver problems or low blood counts. Involvement of the brain is seen in rare instances. This disease has no relationship to systemic lupus erythematosus and is treated very differently.
Lupus is much more common in women (nine out of 10 diagnosed cases are women). This could be caused by the increase of hormones (estrogen in particular) that happens during a woman’s reproductive years. Lupus is also more common in certain ethnicities. African-American, Hispanic, Asian and Native American women, in particular, are most commonly diagnosed with lupus.
What age does lupus (systemic lupus erythematosus) typically start in children?
In most cases, children with lupus develop the condition during their teen years. It most commonly happens when the child goes through puberty (around age 12 on average). This could be due to a possible link between lupus and reproductive hormones that escalate in the body at that time.
Symptoms and Causes
What causes lupus (systemic lupus erythematosus) in children?
The exact causes of lupus are unknown. In general, people have genetic factors that cause the immune system to become overactive. However, not everyone with a genetic predisposition will develop lupus. You may have signs in your genetic code that a condition like lupus will appear sometime in your life and it never shows up.
Lupus can also be triggered by several other factors, including:
- Sun exposure.
- Drug reaction.
- Exposure to cigarette smoke.
- Change in hormones during puberty.
What are the signs and symptoms of lupus (systemic lupus erythematosus) in children?
There are many different symptoms of lupus. Children with lupus may experience symptoms like:
- A red rash on the cheeks and bridge of the nose. This happens in about one-third of the diagnosed cases of lupus.
- A disc-shaped rash (discoid lupus) with raised patches. There could also be scarring from previous outbreaks on the skin.
- A skin rash due to sun exposure.
- Arthritis in two or more joints, often fingers and toes.
- A build-up of fluid surrounding the heart or lungs.
- Kidney problems.
- Low red blood cell (anemia), platelet or white blood cell counts.
Children with lupus will also have abnormal antibody results and high readings for anti-nuclear antibodies (ANA) that can be a sign of an autoimmune disease.
Diagnosis and Tests
How is lupus (systemic lupus erythematosus) in children diagnosed?
If your child has experienced any of symptoms of lupus, reach out to your healthcare provider. It can sometimes be challenging to diagnose lupus because many of the symptoms can overlap with symptoms of other medical conditions. These symptoms can happen over a long period of time or all at once. If the child has had four or more of the signs and symptoms of lupus, it could lead to a diagnosis.
Other factors that a healthcare provider might consider when diagnosing your child with lupus can include:
- Family history of lupus or other autoimmune diseases.
- Age at which the signs and symptoms of lupus happened in the child.
- How long the symptoms have been present.
- What the healthcare provider found during a physical exam.
Management and Treatment
How is lupus (systemic lupus erythematosus) in children treated?
Currently, there’s no cure for lupus. Your healthcare provider will focus on managing the symptoms and the condition over time. The main goal of treatment is to ease your child’s lupus symptoms and prevent their immune system from attacking vital organs. Over time, lupus can cause permanent damage to organs like the kidneys, heart, lungs and brain.
When treating children, there’s usually a team of specialists who all work together on a care plan. These specialists can include a pediatric rheumatologist, nurses, counselors, physical therapists and other medical specialists. Each of these caregivers will work to manage the child’s physical and psychological health.
Counseling may be a necessary part of your child’s treatment plan. This can help your child or teen deal with the fact that they have lupus and teach them how to manage the disease over the long term. Unfortunately, lupus is a life-long disease that will need constant treatment and regular visits with the healthcare team.
The type of medication your child’s healthcare provider will recommend often depends on the severity and types of symptoms your child is experiencing. Medications that are used to treat lupus in children can include:
- Corticosteroids (prednisone) to manage inflammation.
- Hydroxychloroquine (Plaquenil®), an anti-malaria drug used to manage disease flare-ups.
- Immunosuppressants such as azathioprine (Imuran®), mycophenolate mofetil (Cellcept®), methotrexate, cyclophosphamide (Cytoxan®), rituximab (Rituxan®) and Belimumab (Benlysta®).
- 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 lupus (systemic lupus erythematosus) in children?
The main goal of lupus treatment is to put the disease into remission (no active symptoms). When the disease is in remission, your child’s symptoms may stop or be less noticeable. Getting lupus into remission can also help prevent organ damage and improve the general quality of your child’s life. Lupus can sometimes have flares — a sudden worsening of symptoms — so it’s important that your child closely follows the treatment plan and regularly visits the healthcare provider.
Prednisone is one of the fastest-acting and most effective treatments for managing active inflammation in lupus. However, long-term use can lead to serious side effects. These include suppressed immune system, weight gain, stretch marks, high blood pressure, thin bones (osteoporosis), depression, elevated blood sugar (diabetes), glaucoma and cataracts. These conditions can have a big impact on your child's appearance, psychological well-being, and normal growth and development. Because of these factors, it’s important for your child to see their healthcare provider on a regular basis to keep track of these side effects.
Hydroxychloroquine may, in very rare cases, cause permanent damage to the retina. All children taking this medication should have regular checkups with an eye doctor.
Other medications, such as azathioprine, mycophenolate mofetil, and cyclophosphamide, can decrease the number of white blood cell count or cause inflammation in liver. Regular blood tests will be used to watch for this side effect.
Rituximab and belimumab reduce the activity of certain immune cells, called B-cells, which are part of the body’s normal immune system to fight invading organisms. Although these drugs calm the overactive immune system that is seen in systemic lupus erythematosus, they may increase the risk of infection. Your child’s provider will closely monitor these drugs.
Can lupus (systemic lupus erythematosus) in children be prevented?
Because the causes of lupus are not fully understood, there is no way to prevent the condition. You can manage the disease and avoid things that trigger flare-ups in your child.
A few ways to avoid flare-ups of lupus in your child can include:
- Avoiding sun exposure. Wearing sunscreen and protective clothing when your child goes outside can help protect the skin.
- Getting enough sleep (seven to nine hours each night).
- Taking all medications as prescribed.
Outlook / Prognosis
What is the long-term outlook for children who have lupus (systemic lupus erythematosus)?
Children who receive proper treatment for systemic lupus erythematosus generally do very well. Regular management of the child’s symptoms can greatly improve their quality of life.
Children with lupus may have a greater risk for early atherosclerosis (clogging of the arteries) and heart disease when they become adults.
Other issues that may affect treatment and outcomes can include:
- Failing to take medicines or not following the treatment plan.
- Having problems with the nervous system.
- Developing infections.
- Developing kidney disease.
- Having difficulty accessing the healthcare system to receive proper care.
Can my child live a normal life with lupus (systemic lupus erythematosus)?
Children with lupus should live as normal of a life as possible. They should attend school, play sports, participate in extra-curricular activities, and be around friends and family. There are no specific limitations to physical activities for a child with lupus. Outdoor activities can be done, but it is important to protect your child’s skin with sunscreen and skin-covering clothing. Exercise does not harm a child with lupus — in fact, it increases muscle strength and endurance.
There’s no special diet that has been shown to help children with lupus. It is always recommended to maintain a well-balanced and healthy diet.
Is there a support group for lupus (systemic lupus erythematosus)?
The Lupus Foundation is a national non-profit organization that is dedicated to addressing the needs of adults and children who live with systemic lupus erythematosus in the U.S. There are many local offices of the Lupus Foundation throughout the U.S. Many local and national events are organized to educate and connect patients/families together.
More information on the Lupus Foundation can be found at https://www.lupus.org.
A note from Cleveland Clinic
Lupus can be a challenging disease. It can be difficult to diagnose and the symptoms can disrupt daily life. If your child has been diagnosed with lupus, talk to your healthcare provider about ways to help improve their quality of life. Counseling can often be an important part of a child’s treatment plan because lupus is a life-long disease. Working closely with your child’s provider can often help make your child’s life as normal as possible.
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