Childhood-Onset Lupus: Causes, Symptoms, and More

Systemic lupus erythematosus (SLE) is an autoimmune disease that can be seen in children as well as adults. In children, it is called childhood-onset lupus, pediatric SLE, or juvenile lupus. SLE causes systemic (entire body) inflammation that eventually results in damage to joints, skin, and organs. The condition can range from mild to severe, even life-threatening and fatal. The disease is known for causing flare-ups (periods of high disease activity) and periods of remission (little or no disease activity).

Here is what you need to know about childhood-onset lupus, including causes, signs and symptoms, diagnosis, treatment, and complications.

Childhood-Onset Lupus Symptoms

Lupus symptoms are chronic (life-long), but symptoms will become more or less severe during certain periods.

Common symptoms of lupus experienced by children include:

• A rash shaped like a butterfly usually on the bridge of the nose and the checks (malar rash)
• A raised skin rash on the head, arms, back or chest (discoid rash)
• Fever
• Joint pain and swelling
• Mouth sores
• Hair loss
• Fluid in the lungs, heart, or other organs
• Kidney problems (evident in abnormal urine tests)
• Problems with the blood such as anemia, easy bruising, low blood platelets, low white blood cells
• Seizures and other nervous system issues
• Raynaud’s phenomenon, a condition in which blood vessels of the fingers and toes feel numb and cold with certain triggers, such as stress, illness, and cold
• Fatigue and low energy
• Weight loss and decreased appetite
• Swollen lymph nodes
• Photosensitivity, in which a child is especially sensitive to ultraviolet (UV) light, a type of radiation in sunlight and artificial lighting. Sunlight exposure may cause a rash, fatigue, joint pain, and swelling.

Incidence

About 10% to 20% of people diagnosed with lupus are children. Childhood-onset SLE is rare with an incidence of around 0.3 to 0.9 cases per 100,000 children-years and prevalence is 3.3 to 8.8 per 100,000, this according to one report in the journal, Pediatric Clinics of North America. Further, there are more cases of childhood-onset lupus reported in children of Asian, African American, Hispanic, and Native American backgrounds.

Other types of autoimmune arthritis are more common than childhood-onset lupus. The average age of onset is around 11 to 12 years of age. It is rare in children under 5, and as with adult lupus, up to 80% of children affected are girls.

Causes

Exact causes of lupus in both adults and children are unknown. What researchers do know is that lupus is an autoimmune disease. An autoimmune disease is a condition where the immune system mistakenly attacks the body’s own healthy tissues.

Researchers do not yet know why some children develop lupus and why others do not. The condition is not contagious, which means you cannot catch it from anyone.

If you have a parent or sibling with the condition, it is possible you may develop the condition. Having a sibling with lupus means that your risk for developing SLE is 29 times higher than in the general population. However, genes alone are not enough to cause lupus.

Researchers think lupus is caused by a combination of underlying factors, including:

• Environmental: Researchers have identified certain environmental triggers associated with lupus, including stress, smoking, and toxin exposure.
• Genes: According to the Lupus Foundation of America, scientists have identified more than 50 genes associated with lupus. People with one or more of these genes have a higher risk of developing the disease.
• Hormones: Studies in adults have shown abnormal levels of sex hormones, including estrogen, which can contribute to lupus. Lupus is also known for presenting during puberty—the process of physical changes through a child’s body as it matures into an adult body capable of sexual reproduction. Lupus risk is thought to increase at puberty due to the role of sex hormones.
• Infections: Infections, including viruses, bacteria, parasites, and fungus have been linked to lupus. Cytomegalovirus and Epstein-Barr infections are commonly associated with lupus.
• Medications: Lupus can also be drug-induced. According to the Lupus Foundation of America, the most commonly connected medications to drug-induced lupus are hydralazine (used to treat high blood pressure), procainamide (for treating irregular heart rhythms), and isoniazid (for treating tuberculosis). Of course, not everyone who takes these medications will develop lupus.

Risk Factors

There are certain types of people who have an increased risk of lupus. Risk factors associated with lupus are:

• Sex: Women are more likely to develop lupus than are men. However, the disease tends to present more severely in men.
• Age: While lupus can affect anyone of any age, it is most often diagnosed in people ages 15 to 44.
• Race: Lupus is more common in certain races, including African Americans, Asian Americans, Hispanics and Latinos, Native Americans, Native Hawaiians, and Pacific Islanders. In those groups, lupus can develop at earlier ages and can be more severe.
• Family history: Having a family history of lupus means a person’s risk for developing lupus is increased.

Having risk factors for lupus does not mean a person will get lupus. It only means that person’s risk for the condition is increased compared to others without risk factors.

Diagnosis

There is no single test for diagnosing childhood-onset lupus. Doctors usually rely on a series of testing methods, including medical and symptom history, physical examination, bloodwork, and imaging.

Tell your child’s doctor about symptoms and other problems your child may be experiencing. Keep track of symptoms by writing them down, including when they happen and how long they last. Be sure to share if lupus and other autoimmune diseases run in the family.

Your child’s doctor will complete a physical exam, which will look for skin rashes and other signs that something is wrong.

Additional testing may include:

• Blood tests that detect autoantibodies, such as antinuclear antibodies (ANA). ANA is present in up to 98% of people with lupus, according to the Johns Hopkins Lupus Center. ANA is often present at high levels in people who have the condition. However, ANA tests are not specific because while high ANA levels can be present in children with lupus, a positive ANA can also be found in children who are healthy or have other autoimmune diseases.
• Blood work and urine tests to assess kidneys
• A complement blood test to measure the level of complement—a group of proteins found in the blood that help destroy foreign substances and prevent infections. Low levels of complement in the blood are an indication of lupus.
• Blood tests to measure inflammation levels, as a result of inflammatory proteins, including erythrocyte sedimentation rate (also called ESR or sed rate) or C-reactive protein (CRP).
• Imaging, including X-rays of internal tissues, bones, and organs

The American College of Rheumatology has established a set of criteria to assist doctors in making a childhood-onset lupus diagnosis. A child must have at least four of 11 specific elements to be diagnosed with lupus.

These are classified as the following list, with several categories having two or more criteria:

• Malar rash
• Discoid rash
• Sunlight sensitivity
• Mouth sores
• Joint inflammation
• Heart or lung involvement
• Kidney problems
• Seizures or other neurologic issues
• Positive blood work

Treatment

Treating lupus in children has three goals. They are:

• Controlling symptoms, especially joint pain and inflammation
• Slowing down or stopping the immune system from attacking its own healthy tissues
• Protecting the organs from damage

Additionally, your child’s doctor will make decisions for treatment based on age, overall health, medical history, the extent of symptoms, and the effect of the disease on the organs. The doctor will also take into consideration the child’s tolerance for medications and other therapies and parents’ expectations, opinions, and preferences.

If lupus symptoms are mild, treatment may not be necessary. However, your child’s doctor may recommend nonsteroidal anti-inflammatory drugs (NSAIDs) to treat joint pain and manage inflammation. 

Other treatments for childhood-onset lupus may include:

• An immunosuppressive drug called hydroxychloroquine to ease symptoms
• Disease-modifying drugs, like methotrexate, to suppress the body’s autoimmune response
• Corticosteroids to control inflammation
• Drugs called monoclonal antibodies, such as Benlysta (belimumab) and Rituxan (rituximab), which target specific immune cells (Rituxan use is off-label, meaning it is not specifically approved by the Food and Drug Administration for use in this condition)
• Liberal use of sunscreen, decreased time outdoors, especially during the hours where the sunlight is brightest, and wearing hats and long sleeves outdoors
• Getting enough sleep, usually eight to 10 hours of sleep at night
• Stress reduction
• A healthy well-balanced diet
• Immediate treatment for infections

Your child will need frequent monitoring by a rheumatologist to make sure lupus is well-controlled and medications are working and not causing serious side effects. If lupus affects any organs, your child may need to see other specialists. This could include a nephrologist who treats kidney problems, a pulmonologist to treat lung diseases, or a dermatologist to treat skin symptoms, among other specialists.

Lupus Flares

Even with lupus treatment, which can prevent symptoms, a child may still experience flares—periods where symptoms worsen, making the child feel sick. Sometimes, your child will have a warning a flare is coming, and other times they will not. Warning signs may include feeling more tired than usual, a rash, pain, fever, stomach troubles, dizziness, and headaches.

Additionally, there are different things that can trigger a flare-up of symptoms, including exposure to UV radiation, stress, not sleeping well, a minor illness, certain medications, and not taking lupus medications.

Lupus flares range from mild to severe. Some flares may only cause a rash or some joint pain, while others can result in damage to internal organs. If your child is experiencing a lupus flare, call their doctor and ask about getting it under control.

Complications

There are a variety of complications associated with lupus that may affect both adults and children with the condition. Complications tend to be caused by ongoing inflammation and may include problems with:

• Kidneys: Inflammation from lupus can cause kidney damage. It can also lead to kidney failure.
• Blood vessels: Lupus can cause vasculitis, or damaged blood vessels, as a result of chronic and ongoing inflammation. Lupus can also cause bleeding and blood clotting problems.
• Heart: Ongoing inflammation of the heart and surrounding tissues may increase the risk of heart disease, heart attack, or stroke.
• Lungs: Inflammation of the lungs from lupus can lead to breathing problems.
• Nervous system: If lupus inflammation affects the brain, a person can experience headaches, dizziness, and seizures.

People with lupus also have an increased risk of infections. This is not only a byproduct of the disease itself but also of the medications used to treat it that suppress the immune system.

A Word From Verywell

While there is no cure for childhood-onset lupus, the outlook for lupus is generally good. In fact, in most cases, it can be well-managed with lifestyle changes and medications. Therefore, it is a good idea for a parent to learn everything they can about lupus, to work closely with their child’s healthcare team, and to help their child cope and live a normal life. Going to school, exercising, spending time with friends, having a healthy diet, and family activities are all important for a child with lupus.

But having any chronic health condition is stressful and frightening. Your child may become sad, depressed, or upset as a result of having the condition and its effects on their life. Recognize your child’s feelings and allow them to talk about those. Seeking out pediatric and family counseling can also be helpful.