Systemic lupus erythematosus (SLE) is a long-term, autoimmune disease. This is the most common type of lupus. It is what most people are referring to when they use the term “lupus.”

Normally, the immune system protects the body by making antibodies to fight off different things. These can include viruses, bacteria and other foreign substances. With lupus, an abnormal immune system makes antibodies that attack healthy tissue. This leads to pain and inflammation. Lupus can affect the skin, joints, kidneys, brain and other organs.

Between one and a half and two million Americans have some form of lupus. This includes 5,000 to 10,000 children, according to Lupus Foundation of America estimates. Lupus can be mild to severe in children. Children are more likely to have kidney and brain involvement than adults.

Women make up 90% of lupus cases. It can occur at any age. Mostly, it is diagnosed in young women ages 15 to 44. In the U.S., lupus occurs more among African Americans, Hispanic-Latinos and Native Americans. African Americans and Hispanic-Latinos tend to develop lupus at a younger age than white Americans. They also have more severe symptoms.

Lupus can be managed well for most children. Most kids who live with the disease can lead healthy and active lives. The key is early diagnosis, appropriate treatment and preventive therapies.

Pediatric Lupus Causes

The cause of lupus remains unknown. It is not contagious. Some factors may work together to trigger this disease, including:

• Genes. About half of people who have lupus also have a close relative who has an autoimmune condition. A close relative means a mother, father, sister or brother. No single lupus gene has yet been found.
• Hormones. A link to hormones could be why lupus occurs more often in females. It most often starts between age 15 to 44. Also, pregnancy may cause lupus symptoms to flare.
• Environment. Some factors are already known to make lupus symptoms worse. These include exposure to sunlight.

Other factors that may contribute to lupus include:

Some medications can produce lupus-like symptoms. These include medicines for acne and blood pressure. When a person stops taking them, the symptoms go away. This is what makes these cases different than the usual lupus.

Pediatric Lupus Symptoms

Symptoms of lupus can come and go. They can range from mild to severe. Some of the most common symptoms include:

• Butterfly-shaped rash on the cheeks and nose
• Extreme fatigue
• Muscle aches
• Pain in the chest or joints
• Fever
• Color changes or swelling in hands and feet
• Headaches
• Hair loss
• Sores in the mouth or nose
• Weight loss

Sometimes serious problems with blood and blood vessels occur. These include blood clots and anemia (low number of red blood cells). Fingers and toes may turn white or blue. They may feel numb when a person is cold or stressed.

About 20% of children who have lupus will have kidney problems or brain issues. Urine may turn the color of cola or tea. Brain changes may include headaches, depression, anxiety, psychosis (the child loses contact with reality) and seizures.

Pediatric Lupus Diagnosis

There is no single test for lupus. A rheumatologist is a doctor or advanced nurse practitioner who specializes in autoimmune diseases and arthritis. They will start by asking about symptoms. The rheumatologist will also perform a physical exam. Then, they may order lab tests, such as:

• Blood tests to look for low blood counts, anemia or other signs of organ involvement
• Urine tests to check for blood or abnormal protein in the urine
• Antinuclear antibody (ANA) screening blood test for lupus. This test looks for certain antibodies to help diagnose lupus. All patients with lupus test positive for ANA. But not all patients with the antibodies will get lupus. There may be other antibodies specific to lupus that your rheumatologist will look for.

Pediatric Lupus Treatment

The goal of treatment is to manage symptoms like fatigue, pain and inflammation. Medicines work to shut down a child’s overactive immune system. A second goal is to prevent long-term damage to body systems and quiet the disease. Lupus has many different symptoms, so there are many medicines to treat it. The most common medicines to treat lupus include:

• Anti-inflammatories (steroids) to help with inflammation and pain.
• Antimalarials to protect skin from rashes and ultraviolet (UV) light in sunlight. An example is hydroxychloroquine.
• Disease-modifying anti-rheumatic drugs (DMARDs) to decrease joint pain and swelling by quieting the immune system. DMARDs also help prevent joint damage long term.
• Biologics targeted to certain proteins to keep the immune system from attacking the body.
• Anticoagulants to help prevent blood clots when appropriate if patients have certain risk factors or history of a clot. An example is aspirin.

To help prevent flare-ups of lupus, a person can:

• Take all medications as prescribed
• See a rheumatologist to help manage your lupus
• Limit exposure to sunlight
• Use sunscreen regularly
• Reduce the risk of infections
• Get vaccines as recommended
• Get plenty of rest
• Maintain a healthy diet
• Exercise moderately, when possible
• Choose not to smoke
• Develop healthy coping skills and a good support system

For young women who are pregnant, rheumatologists and obstetricians should work together to co-manage lupus. Controlling disease symptoms gives the best chance for a healthy pregnancy.

Pediatric Lupus Long-Term Outlook

Many children will have only mild symptoms. Others will have to take medicine for a lifetime. Most children will lead healthy lives, especially if they have mild to moderate disease.

Science is helping to improve the outlook for children (and adults) with lupus.

Targeted biologic therapies, in use since 2011, have also led to improved treatment options and outcomes. They help prevent symptoms and complications of lupus.

Managing lupus takes a team effort with doctors, patients and families. The Lupus Center at Cincinnati Children’s is home to specially trained doctors who treat lupus in children. Our team is prepared to care for your child’s unique needs. We care for patients from diagnosis to treatment to long-term follow-up. Having this well-coordinated care in one location can improve outcomes.