Lupus and the Nervous System

posted in neuropsychiatric lupus on August 20, 2024 by

Gavin Abson

Updated January 10, 2025

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Systemic lupus erythematosus (SLE) commonly affects the body’s nerves. Our nerves cause us to be able to feel things, move, taste, hear, smell, see, think, walk, and balance ourselves. When lupus affects the nervous system, any of these can become problematic, such as having difficulty feeling things, moving, and remembering things. Lupus, a complex autoimmune disease, affects multiple body systems, including the nervous system. Understanding the relationship between lupus and the nervous system is crucial for diagnosis and treatment. This article explores the impact of lupus on the nervous system, referencing “The Lupus Encyclopedia,” specifically chapter 13, “The Nervous System.”

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“The Lupus Encyclopedia” is published by Johns Hopkins Press, a nonprofit publishing house. Much of the proceeds from book sales goes towards projects like “Project Muse.”

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This blog post article was edited and contributed to by Donald Thomas, MD; author of “The Lupus Encyclopedia.” Parts of this blog post come from “The Lupus Encyclopedia: A Comprehensive Guide for Patients and Health Care Providers, edition 2“

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Understanding Lupus and Its Impact on the Nervous System

Lupus, or SLE, is an autoimmune disease where the immune system attacks healthy tissues. This can cause inflammation and damage in various parts of the body, including the nervous system. The nervous system consists of the central nervous system (CNS) and the peripheral nervous system (PNS).

The Central Nervous System (CNS) and Lupus

The CNS includes the brain and spinal cord. When lupus affects the CNS, called “CNS lupus,” it can lead to several neuropsychiatric syndromes. According to “The Lupus Encyclopedia,” the American College of Rheumatology (ACR) in 1999 created a list of neuropsychiatric syndromes. These syndromes help doctors standardize treatments and research by describing patients similarly.

“In 1999 the American College of Rheumatology (ACR) created a list of neuropsychiatric syndromes (neuro-­ referring to the nerves and “psychiatric” referring to psychiatric conditions) in order to help doctors describe their patients similarly to standardize treatments and research. When SLE patients have any of these problems defined by the ACR, they are said to have neuropsychiatric systemic lupus erythematosus (NPSLE). The different types of NPSLE defined by the ACR are separated into those that affect the CNS and those that affect the PNS. Psychiatric conditions (such as depression and anxiety disorder) are included because they are disorders related to abnormalities in brain nerves.”

The Lupus Encyclopedia (2nd ed.), p. 262

This blog post will focus on the peripheral nervous system. Learn more about CNS lupus in my other blog article: lupusencyclopedia.com/cns-lupus

Neuropsychiatric Systemic Lupus Erythematosus (NPSLE)

NPSLE encompasses various syndromes that affect the CNS and PNS. Symptoms can range from mild cognitive dysfunction to severe neurological issues. Understanding these symptoms is vital for early diagnosis and effective treatment.

CNS Manifestations of Lupus

When lupus affects the CNS, it can lead to several conditions, including:

• Cognitive Dysfunction: Patients may experience memory problems, difficulty concentrating, and confusion. This is often called “lupus fog.” These cognitive issues can impact daily life and job performance.
• Headaches: Migraines and tension headaches are common in lupus patients. These headaches can be severe and persistent.
• Seizures: Lupus can cause seizures, which may vary in frequency and severity. Proper management is crucial to prevent complications.
• Psychiatric Disorders: Depression, anxiety, and mood disorders are prevalent among lupus patients. These conditions can significantly affect quality of life.

This blog post will focus on the peripheral nervous system. Learn more about CNS lupus in my other blog article: lupusencyclopedia.com/cns-lupus

PNS Manifestations of Lupus

Understanding Peripheral Nervous System (PNS) Disorders in Lupus

The peripheral nervous system (PNS) connects the brain and spinal cord to the rest of the body. It consists of two main parts:

1. Somatic Nervous System: Controls voluntary movements, like lifting a cup.
2. Autonomic Nervous System: Manages involuntary functions, like blood pressure, heart rate, and your pupils dilating in the dark.

Lupus can impact the PNS in various ways, but other factors like diabetes, toxins, medications, and infections can also contribute. Diagnosing PNS issues often requires blood tests, EMG with nerve conduction studies, or lumbar punctures.

The rest of this blog post focuses on the peripheral nervous system. See my other post regarding CNS lupus.

Somatic Nervous System Disorders

Guillain-Barré Syndrome (GBS)

This autoimmune condition damages the myelin sheath around nerves, leading to weakness in the legs, arms, and face. It can also affect autonomic functions like blood pressure. Key symptoms include reflex loss and, in severe cases, difficulty breathing. Treatment involves plasmapheresis or IVIG. While most people recover, chronic cases (CIDP) may require long-term treatment.

Mononeuropathy and Mononeuritis Multiplex

• Mononeuropathy: Affects one nerve, often due to lupus-related inflammation. Carpal tunnel syndrome (CTS) is a common example.
• Mononeuritis Multiplex: Involves multiple nerves, potentially causing issues like foot drop. Typically caused by vasculitis, it’s treated with immunosuppressants or IVIG.

Myasthenia Gravis (MG)

Though distinct from lupus, 1% of lupus patients also have myasthenia gravis. It causes muscle weakness, drooping eyelids, and difficulty chewing. Diagnosis involves antibody tests and EMG studies. Treatment may include immunosuppressants and thymectomy, with close monitoring being crucial.

Cranial Neuropathy

Damage to cranial nerves can result in issues like facial weakness (Bell’s palsy), double vision, or hearing loss. These conditions are often caused by nerve inflammation and treated with immunosuppressants. If hearing or vision loss occurs suddenly, it’s essential to rule out other causes like infections or vasculitis.

Polyneuropathy

This condition affects multiple peripheral nerves, starting with the feet. Symptoms include burning pain, numbness, and balance issues. Numbness typically occurs in a “stocking-glove pattern.” Causes can include lupus inflammation, diabetes, or medication side effects. Treatment ranges from addressing underlying causes to using medications like gabapentin for nerve pain.

Many doctors call polyneuropathy by the term “peripheral neuropathy.” However, as we see, it is only one of several different peripheral neuropathies.

Autonomic Nervous System Disorders

Dysautonomia affects involuntary functions like heart rate, blood pressure, pupil dilation, and digestion. Symptoms vary widely, from lightheadedness and heart palpitations to digestive issues. Diagnosis often requires tests from specialists, and treatment focuses on managing symptoms. Postural orthostatic tachycardia syndrome (POTS) is one example. IVIG can be helpful for some cases.

Small-Fiber Neuropathy

This involves damage to small nerves, causing burning pain in the hands and feet. It can affect both somatic and autonomic functions. Diagnosis may require a small fiber neuropathy skin biopsy, and treatment focuses on pain relief with medications like pregabalin and gabapentin.

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Diagnosing NPSLE

Diagnosing NPSLE can be challenging due to the wide range of symptoms. Doctors rely on clinical evaluation, patient history, and diagnostic tests. These tests may include MRI scans, CT scans, and lumbar punctures (spinal taps) to assess CNS involvement. Electromyography (EMG) and nerve conduction studies help evaluate PNS involvement.

Treating NPSLE

Treatment for NPSLE aims to manage symptoms and reduce inflammation and stop nerve damage (if possible). Treatment plans are tailored to individual patients based on the severity and type of symptoms. The 2023 EULAR SLE management guidelines provides some advice on how to treat some NPSLE problems.

Medications

Several medications are used to treat NPSLE:

• Corticosteroids: These drugs reduce inflammation and immune system activity. They are often prescribed for severe symptoms.
• Immunosuppressants: Medications like azathioprine and mycophenolate mofetil suppress the immune system to prevent it from attacking healthy tissues.
• Blood thinners: These drugs thin out the blood. Doctors prescribe them when blood clots are the cause of NPSLE. Examples include aspirin, warfarin, and heparin. They are most often used in patients who are positive for antiphospholipid antibodies.
• Antimalarials: Hydroxychloroquine is commonly used to manage lupus symptoms. It helps control inflammation and prevent flare-ups.
• Anticonvulsants: These drugs are prescribed for patients with seizures to control and prevent seizure activity.

Lifestyle Modifications

In addition to medications, lifestyle changes can help manage NPSLE symptoms:

• Stress Management: Reducing stress through techniques like meditation, yoga, and practicing mindfulness, like deep breathing, can improve overall well-being.
• Regular Exercise: Physical activity helps maintain mobility, reduce pain, and improve mood.
• Healthy Diet: A healthy, balanced anti-inflammatory diet rich in fruits, vegetables, omega-3 fatty acids, probiotics, prebiotics, and lean proteins supports overall health and immune function.

Research and Future Directions

Ongoing research aims to better understand the relationship between lupus and the nervous system. Scientists are exploring new treatments and potential cures. Advances in imaging techniques and biomarkers may improve the diagnosis and monitoring of NPSLE.

Current Studies

Several studies are investigating the effectiveness of new medications and treatment approaches. These studies aim to provide better options for managing NPSLE symptoms and improving patients’ quality of life.

Potential Breakthroughs

Research into the genetic and environmental factors contributing to NPSLE continues. Identifying specific triggers and risk factors may lead to more targeted treatments and preventative measures.

Living with NPSLE

Living with NPSLE can be challenging, but proper management can significantly improve the quality of life. Patients should work closely with their healthcare team to develop an individualized treatment plan. Support from family, friends, and support groups can also make a significant difference.

Support Resources

Several organizations provide resources and support for lupus patients, including:

• Lupus Foundation of America: Offers educational materials, support groups, and advocacy for lupus patients.
• American College of Rheumatology: Provides information on research, treatments, and clinical guidelines for lupus care.

Understanding the impact of lupus on the nervous system is crucial for effective diagnosis and treatment. Neuropsychiatric systemic lupus erythematosus (NPSLE) encompasses a range of conditions that can affect the CNS and PNS. Early diagnosis, appropriate treatment, and lifestyle modifications can help manage symptoms and improve quality of life. Ongoing research continues to explore new treatments and potential cures, offering hope for lupus patients worldwide.

For more in-depth information on Lupus and the Nervous System:

Read more in The Lupus Encyclopedia, edition 2

Look up your symptoms, conditions, and medications in the Index of The Lupus Encyclopedia

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