Neuropsychiatric lupus is a form of lupus that affects the central nervous system. Symptoms may include mood disorders, cognitive impairment, and seizures.

Neuropsychiatric systemic lupus erythematosus (NPSLE) is a subtype of systemic lupus erythematosus (SLE), or lupus, that causes neurological and psychiatric symptoms.

This article looks at what NPSLE is, as well as its symptoms, causes, diagnosis, treatment, and outlook.

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NPSLE is a severe form of lupus and may cause cognitive impairment, mood disorders, and seizures. People may also refer to NPSLE as central nervous system lupus.

According to a 2019 article, experts do not yet fully understand what causes NPSLE. However, it may occur due to an impaired blood-brain barrier in the central nervous system (CNS).

A blood-brain barrier is a system of blood vessels and tissues that filter harmful substances in the body, preventing them from reaching the brain.

When this is not functioning properly, the body’s own antibodies can attack the CNS, possibly causing neuropsychiatric lupus.

NPSLE symptoms may begin in the early stages of lupus and may affect around 39–50% of people with lupus.

NPSLE causes symptoms relating to the brain, spinal cord, and nervous system, which can vary in severity.

NPSLE may cause the following symptoms:

Lupus may cause NPSLE in around 30% of cases, and people may first experience neuropsychiatric symptoms with the onset of lupus.

Researchers have found certain antibodies that may link to NPSLE. Brain cytoplasmic ribonucleic acid (BC RNA) antibodies are antibodies that interrupt normal brain function.

Researchers also found that BC RNAs may influence pathways in the brain that affect cognition, causing some of the symptoms that appear with NPSLE.

Autoantibodies also play a role in NPSLE. Autoantibodies are antibodies the immune system creates to destroy substances that the body produces, and they may contribute to some autoimmune diseases.

As previously mentioned, a disruption in the blood-brain barrier may also contribute to NPSLE, as it allows autoimmune antibodies to enter and attack the CNS.

Vascular problems may also contribute to NPSLE, including reduced or restricted blood flow and thrombosis.

In some cases, organ damage from lupus or lupus medications may cause NPSLE. Doctors refer to this as secondary NPSLE.

According to a 2021 review, NPSLE risk factors may include:

  • genetics
  • presence of certain autoantibodies
  • cytokines
  • accelerated atherosclerosis
  • lupus-related risk factors, such as level of disease activity or duration, heart valve disease, or immune complexes

NPSLE is more common in females, although seizure risk is greater in males than in females.

NPSLE is more common in people of African or Asian descent compared with white people, but the severity of NPSLE is usually greater in white people. Experts are not entirely sure why this is the case.

Currently, there are no specific criteria for diagnosing NPSLE.

Doctors may diagnose the condition by eliminating other possible causes, such as medications or infections.

They may order neurological and psychiatric tests to help them diagnose NPSLE. They may also look at other functions in the body, including cardiovascular health.

Doctors may rely on the following tests and scans to help diagnose NPSLE:

According to a 2021 review, NPSLE is a severe complication of lupus. It may affect a person’s quality of life. Those diagnosed with the condition may have a higher morbidity and mortality risk than people with lupus.

There is currently no standard treatment for NPSLE, but a combination of treatments may help relieve symptoms.

Research is currently investigating the underlying causes of NPSLE to be better able to treat and manage the condition.

There is also a need for more clinical trials to find out the most effective treatments for the condition, as well as new treatment options.

People may need to work with a team of healthcare professionals, including rheumatologists, neurologists, and psychologists, to receive a proper NPSLE diagnosis and to determine the best treatment plan.

Treating NPSLE focuses on relieving symptoms and may vary for each person depending on their specific symptoms.

Treatment may include:

Doctors will also look at treating any underlying conditions that may be contributing to NPSLE, such as chronic inflammation or venous thromboembolism (VTE).

As researchers learn more about NPSLE and its exact causes, including specific antibodies that may play a role in the disease, they can work to develop new treatments that focus on the underlying cause, rather than just treating symptoms.

Researchers are looking into treatments such as biologics and small-molecule inhibitors that target specific inflammatory pathways to treat NPSLE.

NPSLE is a form of lupus that causes neurological and psychiatric symptoms, such as cognitive impairment and mood disorders. NPSLE affects the CNS, which includes the brain and spinal cord.

Treating NPSLE may include a combination of medications and therapies to help manage and relieve symptoms.

Researchers are currently investigating NPSLE to find out more about the exact causes and new treatment options.

If people are interested in trying new treatments, they may want to discuss the possibility of taking part in clinical trials with a healthcare professional.