Lupus and the Central Nervous System

Lupus is an autoimmune disease that affects the immune system. Systemic lupus erythematosus (SLE) is the most common type of lupus. This disease attacks the tissue of different organs throughout the body.  It’s a lifelong illness with a significant number of symptoms and many different clinical presentations.

Along with joint pain, fever, rashes, and fatigue, lupus can cause damage to the body's organs due to chronic inflammation. When lupus affects the brain and spinal cord, it is called central nervous system (CNS) lupus or Neuropsychiatric lupus (NPSLE). About 40% of all SLE patients have clinical involvement in the CNS.

Is CNS Lupus serious?

Neurologic symptoms are a feature of SLE and are considered one of the major manifestations of lupus. Symptoms can range from minor abnormalities to rare and severe presentations. They can be unpredictable, occurring at any time during the disease, but they often appear in the first or second year of a lupus diagnosis.

CNS lupus can limit a person's mental functioning. Cognitive impairment is the most common cause.  This can significantly affect a person’s quality of life.

Symptoms and Diagnosis

CNS lupus can present as mild, non-specific symptoms such as brain fog and mild mood disorders. Headaches are very common in lupus, and migraines can last days or even weeks. 

Over time, new symptoms can develop, or some symptoms may happen less often. CNS lupus is characterized by a variety of neurological and psychiatric symptoms, including: 

·    Confusion and trouble concentrating

·    Psychiatric disorders

·    Generalized and focal seizures

·    Stroke

·    Blood clots

·    Movement disorders

There is no single diagnostic test. It’s important to rule out other possible causes, such as sepsis, drug effects, metabolic disturbances, and severe hypertension. It can be hard to distinguish CNS lupus from other disorders that affect the central nervous system. 

Brain scans may help diagnose CNS lupus. Computed tomography (CT) and magnetic resonance imaging (MRI) scans may observe lesions, infarcts, vasculopathy, and hemorrhages. An electroencephalogram (EEG) can check the electrical activity in your brain. Lab tests look for different markers in your blood. All these tests can help provide a clear diagnosis. 

Causes

Researchers are not sure how lupus causes neuropsychiatric problems. One known theory is that antibodies cause inflammation in the brain. Another theory is that the body releases specific proteins (cytokines), which cause brain inflammation. Lupus may attack the nervous system via antibodies that bind to nerve cells or the blood vessels, interrupting the blood flow to nerves.

Genetic predisposition and environmental factors can play a role in diagnosing CNS lupus. It is not clear why some people with SLE develop neurological symptoms while others do not. More research is needed in this area. 

What are Triggers?

A trigger is something that brings on or worsens symptoms. A trigger of CNS lupus can be very serious. This can take a person's lupus from remission into active disease. Some triggers can include:

·    Viral infection

·    Trauma and stress

·    Childbirth

·    Menopause

·    Puberty

·    Sunlight

·    Foods

A trigger can lead to a flare. A flare is usually the disease awakening and causing systems. Lupus flares can vary in length, from days to weeks or more. Disease remission is the period of time when symptoms have improved.

Complications

CNS lupus can be life-threatening. In rare cases, serious problems, such as psychosis, can occur. People may see and hear things that disconnect them from reality. This condition generally happens within the first three years after a SLE diagnosis. Episodes can coincide with flares or with specific lupus symptoms.

Inflammation of the spinal cord, called myelitis, may lead to paralysis and sensory loss. The risk for ischemic stroke is higher and usually occurs within the first five years of the diagnosis. Identifying a complication associated with CNS lupus and receiving treatment quickly may improve health outcomes. Late diagnosis and poor adherence to therapeutic regimens may cause further complications, including death.

Are Women More Affected than Men?

 SLE can affect people of all ages, including children. Women are most at risk, particularly aged ages 15-44 years. For every one man, there are 4 to 12 women who have lupus.  Lupus flares can happen during pregnancy, most often in the first or second trimester, and to those who have active disease at conception. 

Treatments 

Lupus requires proper care with more than one medical specialty. Specialists and neuropsychologists need to determine whether a person's nervous system problems are related to lupus. You may need to visit rheumatologists, neurologists, or psychiatrists.  

The treatment of CNS lupus depends on the problem and its cause. It can often be challenging to differentiate between symptoms that are due to the disease or other common triggers, like medications and infections.

Medications

Active lupus, or a flare,  is treated with different types of medications. Steroid hormones known as glucocorticoids reduce the inflammatory response in the body quickly. Immunosuppressive drugs reduce symptoms by lowering abnormal immune system activity. Intravenous immunoglobulin  (IVIG) can suppress inflammation by decreasing the production of pro-inflammatory cytokines. These medications can have serious side effects and increase the risk of infection.

Anti-seizure medications may be used for precaution when needed. About 3-20% of patients with SLE may experience a stroke at some point. High-risk groups are usually started on anticoagulant medications to prevent stroke. Antipsychotic therapy is the usual treatment of SLE-related psychosis. Immunosuppressants and corticosteroids lessen the inflammation in the brain. Mood stabilizers and anti-depressant medications are also used in treatment.

Diet and Exercise

Diet and exercise can improve your lupus and overall well-being. Regular exercise can bring physical benefits, like improved mobility. There is no special diet for

lupus, but eating various healthy foods low in fat and sugar can give your body the nutrition it needs to manage your symptoms.

Avoiding Stress

Avoiding stress is vital for someone with lupus. Stress can take many forms, and emotional health and stress management are essential. Journaling to keep track of stress symptoms can help prevent them from getting worse.

Education

The more you know about your disease, the better you can manage your symptoms. Ask for help from your care team. Know how to recognize the symptoms of your lupus disease. The Lupus Foundation of America is a valuable educational resource for those with lupus.

Rest

Rest is needed to restore energy. Prioritizing a healthy sleeping pattern is important.  Most people need 6 to 8 hours of sleep every night. It is also good to pace daily activities and set boundaries if downtime is needed.

Summary

Although there is no cure, CNS lupus is a serious and treatable illness. It is necessary to follow a well-planned and monitored medication regimen. Many areas of NPSLE have not yet been clarified and require further study. Proper medical care, preventive care, and education can significantly improve function and quality of life for someone experiencing CNS lupus.

References

Van Dam, A. P. (1991). Diagnosis and pathogenesis of CNS lupus. Rheumatology International, 11(1), 1–11. https://doi.org/10.1007/bf00290244

Neuropsychiatric lupus: SLE and the brain | HSS Rheumatology. (n.d.). Hospital for Special Surgery. https://www.hss.edu/conditions_neuropsychiatric-sle-lupus-and-brain.asp

 Central Nervous System Involvement - LUPUS UK. (n.d.). LUPUS UK. https://lupusuk.org.uk/medical/lupus-diagnosis-treatment/clinical-aspects-of-lupus/cns-involvement/

 Systemic lupuserythematosus (SLE). (2023, January 31). Centers for Disease Control and Prevention. https://www.cdc.gov/lupus/facts/detailed.html