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Systemic lupus erythematosus (SLE), the most common form of lupus, can affect any part of the body, including the brain, spinal cord, and nerves. Lupus that affects the nervous system is called neuropsychiatric systemic lupus erythematosus (NPSLE), or neuropsychiatric lupus.
NPSLE symptoms vary from person to person and sometimes get worse during lupus flares. One of the most serious complications of NPSLE is seizures, which occur in about 2 percent to 8 percent of people with SLE — and more often in those with neuropsychiatric involvement.
Other possible symptoms include headaches, mood disorders, and brain fog (difficulty with memory, thinking, and concentration).
This article explains what neuropsychiatric lupus is, what may cause it, what symptoms to watch for, and how it’s managed.
It’s unknown exactly how common NPSLE is. There’s no single test or imaging scan to confirm this condition. Instead, doctors diagnose NPSLE by ruling out other possible causes and relying on their clinical judgment — often based on symptoms and test results.
NPSLE symptoms are often vague and overlap with those of other conditions, which also makes it hard to estimate how many people have NPSLE. For example, headache and mild cognitive dysfunction (thinking and memory problems) are common in people with lupus but typically aren’t linked directly to the condition.
Studies show that anywhere from 12 percent to 95 percent of people with lupus may have some nervous system involvement. That wide range highlights how differently NPSLE can affect each person. More serious brain-related symptoms, such as stroke and psychosis (a loss of contact with reality), are less common but require fast medical attention. Severe memory or thinking problems are estimated to affect between 3 percent and 5 percent of people with lupus.
Researchers are still learning why some people with lupus develop NPSLE and others don’t. It likely comes down to a mix of disease severity, genetics, and when this autoimmune disease first appears.
People with more active lupus — those with more symptoms or more inflammation — may be at increased risk. One tool doctors use to measure disease activity is called the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), which is based on symptoms. A higher SLEDAI score means more severe disease, which is linked to a greater chance of NPSLE.
Having certain autoantibodies (immune system proteins that target healthy tissues) such as antiphospholipid antibodies may also raise the risk of NPSLE.
Some genetic mutations (changes) may also increase NPSLE risk. For example, a mutation in the TREX1 gene has been linked to NPSLE with seizures. One study found that people with 10 or more mutations associated with NPSLE had double the chance of developing nervous system symptoms.
People diagnosed with lupus before age 50 are at higher risk of some types of symptoms. For example, psychosis and seizures are more common in early-onset NPSLE. When seizures do occur, they usually happen just once, soon after diagnosis. Long-term or repeated seizures are uncommon.
Scientists believe that many factors contribute to NPSLE. Most of these are linked to inflammation and problems with blood flow in the brain — both of which can be caused by lupus.
Normally, the brain is protected by a shield of cells called the blood-brain barrier. This barrier keeps harmful substances like bacteria, viruses, and certain proteins out of the brain. Once the blood-brain barrier is weakened, immune system cells and proteins that normally stay in the bloodstream can enter the brain and cause harm. Molecules called cytokines, which help fuel inflammation, may also pass into the brain and affect how it works.
The blood-brain barrier may also be weakened by factors such as:
Doctors believe that these factors may lead to some of the symptoms of NPSLE.
Another possible cause of NPSLE symptoms involves thrombosis (blood clots in the brain). These are called thrombotic processes. Some lupus antibodies, such as antiphospholipid antibodies, can get through the damaged blood-brain barrier and form clots. When lupus is really active, more clots can form and damage blood vessels, causing strokes or hemorrhaging (bleeding in the brain).
Sometimes, inflammation and blood clots happen at the same time. This combination can cause a mix of symptoms that make NPSLE hard to diagnose and treat.
NPSLE can cause a wide range of symptoms that affect how a person thinks, feels, or moves. These symptoms may show up around the time of diagnosis, but they can also appear anytime later.
Although not a common symptom of NPSLE, seizures can be serious. Up to 66 percent of people who experience seizures with lupus will have one within the first year after their diagnosis. Younger women are more commonly affected, according to the journal Seizure.
Certain factors raise the risk of lupus-related seizures, including high disease activity, involvement of other organs, and the presence of certain antibodies or genes.
Tonic-clonic seizures are the most common type of seizure in people with lupus. Formerly called grand mal seizures, these seizures usually involve muscle jerking and loss of consciousness.
Symptoms commonly reported by people with lupus include:
NPSLE symptoms such as these are less common:
These rare NPSLE symptoms can be serious and usually require urgent care:
Treatment for neuropsychiatric lupus depends on the likely cause. For inflammation, doctors may prescribe immune-suppressing medications, such as corticosteroids and other immunosuppressants, to calm the immune system and reduce brain inflammation. For blood clots, medications that help prevent blood clots and stroke may be used. These include warfarin, statins, and other anticoagulants (blood thinners).
Some treatments target specific NPSLE symptoms. These treatments include antiepileptic (anti-seizure) medication for seizures, antidepressants for mood disorders, and antipsychotics for psychosis or other severe mental health symptoms.
It’s important to let your doctor know if you’re having any neurological or mental health symptoms. Research shows that many people with lupus don’t mention these symptoms to their healthcare team, and most healthcare providers don’t ask.
Because there’s no simple test for most NPSLE symptoms, open, nonjudgmental communication is key. Sharing your experiences helps your doctor create a treatment plan that works for you and boosts your quality of life.
MyLupusTeam is the social network for people with lupus and their loved ones. On MyLupusTeam, members come together to ask questions, give advice, and share their stories with others who understand life with lupus.
Have you been diagnosed with lupus? Are you wondering if your brain-related symptoms are connected to your diagnosis? Share your experience in the comments below, or start a conversation by posting on your Activities page.
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