What Is NPSLE?

Systemic Lupus Erythematosus (also called SLE or lupus) is an autoimmune disease that can affect nearly any part of a child’s body. “Autoimmune” means the immune system sees the body as something foreign and attacks it. SLE is a lifelong disease that can be severe. It can have periods with quiet spells and flare-ups. It can occur at any age. Up to 20% of all people with SLE are diagnosed in childhood. SLE occurs in two–26 per 100,000 children. Both males and females develop SLE but it is more common in females. People of all ethnicities and races can develop SLE. When SLE affects the brain, spinal cord or other nerves, it is called neuropsychiatric systemic lupus erythematosus (NPSLE). This happens in about 66% of all people who have SLE.

Causes of NPSLE

The cause of NPSLE (and SLE in general) is unknown. Some factors may combine to trigger this disease, such as:

• Genes: About half of people who have lupus also have a close relative who has lupus. A close relative means a mother, father, sister or brother. No single lupus gene has yet been found.
• Hormones: A link to hormones could explain why lupus occurs more often in females. It most often starts between ages 12 to 44.
• Environment: Some factors are already known to make lupus symptoms worse. These include some prescription drugs and exposure to sunlight.

Other factors that may contribute to NPSLE include:

• Stress
• Infections
• Cigarette smoke and secondhand smoke

NPSLE Symptoms

Symptoms of NPSLE may persist or may come and go. They can range from mild to severe. Some of the most common symptoms that may affect the brain and spine include:

• Headaches not improved by over-the-counter medication
• Anxiety
• Depression
• Confusion
• Attention problems
• Seizures
• Stroke
• Abnormal gait (walking)
• Abnormal movements
• Tremors (shaking)
• Psychosis
• Trouble seeing
• Weakness and numbness of the face and / or arms and legs

Some children have “brain fog.” This is a combination of memory loss, fatigue and trouble expressing thoughts.

Blood vessel inflammation (called vasculitis) is a very rare form of NPSLE. It can cause high fevers, seizures and stroke. It can also cause problems with thinking, attention and memory.

NPSLE Diagnosis

There is no single test to diagnose NPSLE. The doctor will start by asking about symptoms, do an exam and get additional tests and imaging. Doctors who treat SLE are rheumatologists. A rheumatologist is a doctor who specializes in autoimmune diseases affecting the joints, muscles, connective tissues, skin, brain and other parts of the body. When NPSLE occurs, rheumatologists and neurologists work together to treat this. A neurologist is a doctor who specializes in disease conditions of the brain and spine. People with NPSLE may have other members of the care team depending on the type, how severe it is and what other areas of the body are affected by SLE. To diagnose NPSLE, the doctor may order lab and imaging tests, such as:

• Blood tests for signs of inflammation and of SLE.
• Magnetic resonance imaging (MRI) to detect brain or spine changes
• Specialized imaging such as magnetic resonance spectroscopy to look for chemical imbalances in the brain.
• Spinal tap to test fluid around the brain and spine for signs of inflammation.
• Electroencephalogram (EEG) to look for seizures. These are abnormal brain electrical activity and can be caused by inflammation.
• Tests for behavior, memory and thinking to study how well a child’s brain is working.

NPSLE Treatment

The most common treatments for pediatric NPSLE include:

• Steroids
• Conventional and biologic immunosuppressive medications
• Antimalarial-like drugs

These medicines are used to calm the immune system. They prevent and control inflammation. Children may take these medicines long-term to keep the immune system quiet.

If NPSLE is severe, most children go to the hospital for treatment. Some children may respond right away to medication, but for other children, it takes a while for the medication to work. People with NPSLE A child should have routine doctor visits to monitor check progress and improvement. 

NPSLE Long-Term Outlook

If NPSLE is treated early, most children have a normal lifespan and good quality of life. When one treatment does not work, doctors will keep trying other options.

The Multiple Sclerosis and Neuroimmunology Center at Cincinnati Children’s is home to specially trained doctors who treat NPSLE in children. Our team is prepared to care for your child’s unique needs, from diagnosis to treatment to long-term follow-up. Receiving this well-coordinated care in one location can improve outcomes.