Lupus in Children

What is lupus in children?

Lupus is an autoimmune disease that healthcare providers diagnose in people of all ages — including kids and adolescents. Providers may use the phrase “lupus in children” to refer to lupus that’s diagnosed before age 18. Although lupus generally affects children and adults in similar ways, children with lupus may have a higher risk of:

• More severe or active disease (increased inflammation and related symptoms).
• Organ damage.
• Needing more aggressive treatments (for example, higher doses of medications).
• Harmful effects later in life from long-term corticosteroid use (a form of treatment).

Systemic lupus erythematosus (SLE) is the most common type of lupus among children and adults. And it’s what most people mean when they talk about lupus in children. Other terms that refer to lupus in children include:

• Childhood-onset lupus, or childhood-onset SLE.
• Juvenile-onset lupus, or juvenile-onset SLE.
• Pediatric lupus.

Most cases of juvenile-onset lupus start around age 12. But the disease can start sooner or later than that. It’s rare for lupus to begin before age 5.

If your child has SLE, that means their immune system — which is supposed to protect them against threats like infections — is attacking their body. Specifically, their immune system is producing abnormal antibodies, called autoantibodies. These autoantibodies attack healthy (“self”) body tissues. This can lead to a wide variety of symptoms and even damage to organs like their heart, lungs, brain, kidneys and skin.

As with any chronic condition, keeping up with treatment is essential. Your child’s healthcare team will help your family understand what treatment will involve and what to expect down the road.

What are the signs and symptoms of lupus in children?

There are many different signs and symptoms of lupus, and the disease affects each person in slightly different ways. Healthcare providers call one’s individual experience their “lupus fingerprint.”

Symptoms are things your child feels or notices in daily life. Signs are things your child’s healthcare provider can see or measure through a physical exam or testing.

Symptoms of lupus in children

Symptoms your child may experience include:

• A rash on their cheeks and the bridge of their nose (“butterfly” or malar rash).
• Fatigue.
• Headaches.
• Fevers.
• Weight loss.
• Hair loss.
• Sores in their mouth or nose.
• Swelling and pain in their joints (arthritis).

These symptoms can happen over a long period of time or all at once.

Keep in mind that when taken separately, each of these symptoms might have many possible causes. Healthcare providers look for the presence of multiple symptoms before considering lupus. It’s rare for a single symptom (like fatigue) to be the only one.

Signs of lupus in children

Children with lupus may have low blood cell counts — specifically, reduced numbers of:

• Red blood cells (anemia).
• Platelets (thrombocytopenia).
• White blood cells (leukopenia).

Low blood cell counts can happen due to inflammation or autoantibody attacks. Or, it may occur as a side effect of some lupus medications.

Children with lupus usually test positive for antinuclear antibodies (ANA). But just because an antinuclear antibody (ANA) test is positive doesn’t mean your child has lupus or another autoimmune disease. Their provider will interpret the results and tell you what they mean.

What are possible complications of lupus in children?

Treatment can greatly lower your child’s risk of complications. But it’s important to know that some children with lupus develop:

• Kidney inflammation and damage (lupus nephritis).
• Inflammation of the lining of their lungs (pleurisy) or the sac surrounding the heart (pericarditis). Sharp chest pain is a common symptom of either condition.
• Central nervous system (brain and spine) inflammation. Signs and symptoms include headaches, behavioral changes and seizures.
• Blood clots, most often in their legs. Signs and symptoms include pain, swelling, warmth and redness/discoloration in the affected area.

What causes lupus in children?

Researchers don’t yet know the exact cause of lupus in children or adults. But they’re looking into a two-hit theory. This means two factors interact to make a person develop lupus:

• Genetics make a person susceptible or predisposed to developing lupus.
• A second factor like hormones, a viral infection or exposure to sunlight (UVA/UVB light) unmasks lupus and starts the disease process.

But not everyone with a genetic predisposition will develop lupus. A person may have signs in their genetic code that a condition like lupus will appear but it never does.

Researchers continue to investigate the causes of lupus.

How do healthcare providers diagnose lupus in children?

Healthcare providers diagnose lupus by:

• Talking to your child (and you) about the signs and symptoms you’ve noticed.
• Doing a thorough physical exam.
• Doing blood and urine (pee) tests to look for markers of inflammation and signs of a possible autoimmune disease (like antinuclear antibodies).

Other factors that a healthcare provider might consider include:

• A biological family history of lupus or other autoimmune diseases.
• The age at which the signs and symptoms of lupus began.
• How long the symptoms have been going on.

If your child has experienced any lupus symptoms, reach out to a pediatrician. Some symptoms, like a fever, can occur for lots of reasons, which are often temporary and harmless. But other symptoms like hair loss or a distinctive facial rash can be red flags that something more serious is going on. Your child’s provider will evaluate their symptoms to reach a diagnosis.

What is the treatment for pediatric lupus?

There’s a lot your child’s healthcare team can do to help your child feel better from day to day and avoid complications. But there’s currently no cure for lupus. Treatment will focus on:

• Easing your child’s symptoms.
• Preventing your child’s immune system from attacking vital organs.
• Managing your child’s overall physical and psychological health.

A team of specialists will work together on a care plan that’s tailored to your child’s needs. These specialists can include:

• A pediatric rheumatologist.
• Nurses.
• Mental health professionals.
• Physical therapists.
• Other medical specialists.

Medications to treat lupus in children

Many different medications treat lupus in children and adults. Your child’s healthcare team will recommend the appropriate medications based on the severity of your child’s lupus (including the extent of organ damage) and their symptoms. Possibilities include:

• Corticosteroids (prednisone) to manage inflammation.
• Hydroxychloroquine (Plaquenil®), an anti-malaria drug used to manage disease flare-ups.
• Immunosuppressants like azathioprine (Imuran®), mycophenolate mofetil (Cellcept®), methotrexate, cyclophosphamide (Cytoxan®), rituximab (Rituxan®) and belimumab (Benlysta®).
• Pain relievers like ibuprofen or naproxen.
• Calcium and vitamin D supplements to prevent osteoporosis (a possible side effect of long-term use of corticosteroids).

What happens after my child’s treatment?

The main goal of lupus treatment is to put the disease into remission (no disease activity) or reach a state of low disease activity (LDA). With remission or LDA, your child has no symptoms or their symptoms are less noticeable, and your child is less likely to have organ damage or need hospitalization.

Lupus can sometimes have flares, or a sudden worsening of symptoms. A flare happens when lupus becomes active again — this means autoantibodies start attacking body tissues.

You might not be able to predict a flare. But your child’s healthcare provider will help you identify possible triggers, and they’ll tell you how often you should come in for appointments. Your child may need follow-up blood tests on a regular basis to check for signs (biomarkers) of disease activity.

Side effects of treatment

Prednisone is one of the fastest-acting and most effective treatments for managing active inflammation in lupus. But long-term use can lead to side effects like:

• Suppressed immune system.
• Weight gain.
• Stretch marks.
• High blood pressure.
• Osteoporosis.
• Depression.
• Elevated blood sugar (diabetes).
• Glaucoma.
• Cataracts.

These side effects can influence your child’s appearance, mental health, growth and development. Because of these factors, it’s important for your child to see their healthcare provider on a regular basis.

Hydroxychloroquine may, in very rare cases, cause permanent damage to the retina. All children taking this medication should have regular checkups with an eye care specialist.

Other medications — such as azathioprine, mycophenolate mofetil and cyclophosphamide — can lower your child’s white blood cell count or cause inflammation in their liver. Your child’s healthcare team will do routine blood tests to watch for these side effects.

Rituximab and belimumab reduce the activity of certain immune cells, called B-cells, which help defend your child’s body against invading germs. Although these drugs calm your child’s overactive immune system — and therefore help treat lupus — they may raise your child’s risk of infections. Your child’s care team will closely monitor these drugs and their effects.

Can lupus in children be prevented?

Because researchers don’t fully understand the causes of lupus, there’s no way to prevent the condition. But you can help your child manage the disease and avoid flare-ups by:

• Making sure your child gets enough sleep.
• Giving your child the medications that their provider prescribes.
• Telling their provider right away about any new or changing symptoms.
• Making sure your child wears protective clothing and sunscreen when they’re outside. This is because sunlight can trigger an immune response that leads to a lupus flare.

What is the outlook for children with lupus?

Your child’s outlook depends on many factors, including:

• The severity of their disease and how much it affects their organs.
• When they start treatment and how their body responds to it.
• Any complications that arise.

Children who receive treatment for lupus generally do very well. Treatment can help ease your child’s symptoms so they can enjoy school and other activities. It’s important to work closely with your child’s healthcare team so you understand exactly what treatment involves and your role in making it happen (for example, when and how to give your child medications).

Children with lupus may have a greater risk for early atherosclerosis (clogging of the arteries) and heart disease when they become adults. You may wish to speak with a pediatric cardiologist about this risk.

How will lupus affect my child’s life?

As a parent, you might worry about how lupus will affect your child’s life. Will it make school more difficult or keep your child from enjoying sports or activities? Will it affect their friendships or social life?

These are common concerns, but for many kids, managing lupus becomes a routine part of life rather than a roadblock. Thanks to advances in diagnosing and treating lupus, children with this condition can enjoy the things that make them happy and pursue their goals just like others their age.

How can I care for my child?

Your child’s healthcare team will tell you how to help your child live with lupus. In general, it’s a good idea to:

• Track your child’s symptoms and possible triggers. Share these with their care team.
• Teach your child about their condition in terms they can understand.
• Have an open-door policy for your child to come to you with questions and concerns. Tell them it’s OK if they’re upset or just want to vent, and that you’re there for them.
• Encourage your child to talk to a counselor, who can help them explore their feelings about living with lupus and develop coping skills.
• Make sure your child wears sunscreen (SPF 30 or higher) every day, even if it’s cloudy. They should also wear protective clothing and hats when they’re outdoors. Limit your child’s time outdoors when the sun is strongest (typically between 10:00 a.m. and 4:00 p.m.).
• Teach your child simple tips for avoiding infections. Lupus and medications that treat lupus may make your child more vulnerable to getting sick. Handwashing and avoiding others who are sick can help protect your child.
• Encourage physical activity. Exercise doesn’t harm a child with lupus — in fact, it increases their muscle strength and endurance. But if your child is fatigued or otherwise not feeling well, encourage them to rest.
• As your child gets older, help them take a more active role in their healthcare. Encourage them to ask their providers questions and to do more of the talking during appointments.
• Talk to administrators at your child’s school to learn about available accommodations.

Remember that in order to take care of your child, you also have to take care of yourself. Caregiver burnout is very real — and it’s hard to avoid when you’re on call 24/7. Make it a point to keep up with your own healthcare appointments. And consider connecting with a support group for parents of children with chronic diseases.

What is neonatal lupus?

Neonatal lupus, or lupus in babies, is a separate condition that happens when certain antibodies pass through the placenta to a fetus during pregnancy. Neonatal lupus has no connection with pediatric lupus. Healthcare providers treat these two conditions very differently.

A note from Cleveland Clinic

Learning your child has lupus might make you feel all sorts of emotions. Shocked, scared, confused, overwhelmed. Take the time to process how you’re feeling and lean on your child’s healthcare team to give you the information and support you need to plan the next steps. We’ve come a long way with diagnosing and treating lupus in children. With treatment and regular follow-ups, your child can enjoy the simple moments — like ice cream in the summer or play dates with their friends — without letting lupus take center stage.

As you’re learning more and caring for your child, don’t forget to take care of yourself, too. It’s easy to put your own health on the backburner when you’re focusing so closely on someone you love. But you can be the best possible caregiver when you’re looking out for your own needs, too. See your provider regularly and carve out some time — even just a few minutes — each day for self-care.