Systemic lupus erythematosus (SLE) is an autoimmune disease. The body’s immune system attacks its healthy cells. When lupus affects the brain and spinal cord and other nerves, it is called neuropsychiatric systemic lupus erythematosus (NPSLE) or central nervous system (CNS) lupus.
Some of the characteristics of NPSLE are:
- Occurs in 40 percent of all people who have SLE
- It is a lifelong disease with in-between spells and flare-ups
- Occurs at any age but is rare in children
It is difficult to identify if the symptoms of NPSLE are due to lupus or other comorbid conditions. For example, headaches can be due to lupus, as well as migraines, tension headaches, and sinus problems.
How is neuropsychiatric lupus caused?
The exact cause of systemic lupus erythematosus (SLE) and the reason why lupus causes neuropsychiatric systemic lupus erythematosus (NPSLE) is unknown. Some of the estimated reasons that various researchers have come up with are:
- Certain autoantibodies reach the cerebrospinal fluid in the brain and cause inflammation, triggering neurological symptoms.
- The release of certain inflammation-associated proteins called cytokines can cause inflammation within the brain.
- Stroke can occur in people who have antiphospholipid antibodies in their blood.
NPSLE could have the same risk factors as SLE. Some of the environmental, genetic, and hormonal factors may contribute to getting lupus.
- Genetic factors: It can play an important role in triggering NPSLE. If a person has a family member with a history of lupus, the person is sure to get SLE and probably NPSLE.
- Hormonal factors: A link to hormones could explain why lupus occurs more often in females. It most often starts between the ages of 15 and 44 years.
- Environmental factors include:
- Other risk factors include:
- Family history of SLE
- Female gender
- Chronic infections
- Use of estrogen in women undergoing menopause
- Vitamin D deficiency
- In pregnancy, however, breastfeeding has shown to decrease the risk of SLE
- Women of childbearing ages (15 to 44 years)
- High-risk ethnic backgrounds are African Americans, Asians, Hispanics, and Caucasians
- Early-life risk factors include:
- Low birth weight (less than 2,500 grams)
- Preterm birth (birth that occurs before the 37th week of pregnancy)
- Exposure to pesticides during childhood
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What are the symptoms of neuropsychiatric lupus?
Systemic lupus erythematosus (SLE) when affecting the brain can cause the following symptoms:
- Headaches not improved by over-the-counter medication
- Attention problems
- Abnormal gait (walking)
- Vision problems
Neuropsychiatric systemic lupus erythematosus (NPSLE) can cause brain fog in some people, resulting in symptoms such as:
The severe form of NPSLE called vasculitis (blood vessel inflammation) can lead to certain symptoms, including:
How is neuropsychiatric lupus treated?
There is no established cure for neuropsychiatric systemic lupus erythematosus (NPSLE). Management of NPSLE depends on the symptoms and their cause.
Hydroxychloroquine has been found to be effective in the long-term treatment of NPSLE. Immunosuppressive medicines help inhibit the activity of the immune system, which can help treat NPSLE.
Cincinnati Children's Hospital. Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) in Children. https://www.cincinnatichildrens.org/health/n/neuropsychiatric-systemic-lupus-erythematosus
Hospital for Special Surgery. Neuropsychiatric SLE: Lupus and the Brain. https://www.hss.edu/conditions_neuropsychiatric-sle-lupus-and-brain.asp
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