Vasculitis in the Nervous System

What is vasculitis in the nervous system?

Vasculitis is inflammation of blood vessels that carry blood through the body. It happens when the body’s immune system attacks the blood vessel by mistake due to an infection, a medicine, another disease, or often, an often unknown cause. Vasculitis can affect blood vessels of any type, size, or location, including those in the central  and peripheral nervous systems that supply nutrients to the brain, spinal cord, and peripheral nerves. It may begin suddenly or develop over time.

Inflammation from vasculitis can cause the walls of blood vessels to weaken, stretch, thicken, and swell or develop scarring, which can slow or completely stop the normal flow of blood. In the brain and elsewhere, the weakened vessel can burst, causing bleeding into surrounding tissues.

Vasculitis can cause strokes, blood clots, swelling, and other conditions that affect the brain, nervous system, and other organs.

Vasculitis in the brain can cause cerebral aneurysms (weak spots on a blood vessel in the brain that balloon out), which can burst and spill blood into the surrounding tissue. This is called a hemorrhagic stroke. Vasculitis can also cause ischemic strokes, which are the most common kind of stroke. Ischemic stroke can happen when blood in the inflamed vessel clots (called thrombosis), blocking blood flow in the brain. 

Symptoms of vasculitis

The symptoms of vasculitis may include:

• Fever
• Malaise (feeling restless and sad)
• Rapid weight loss
• Aches and pains in the joints and muscles
• Pain while chewing or swallowing
• Unusual rashes or skin discoloration
• Problems with the kidneys or other organs
• Transient ischemic attack (TIA, a warning sign for a stroke)
• Headaches, especially a headache that doesn't go away
• Confusion or forgetfulness, sometimes leading to dementia
• Paralysis or numbness, usually in the arms or legs
• Seizures or convulsions
• Abnormal sensations or loss of sensation
• Trouble speaking or understanding
• Vision problems, such as double vision, blurred vision, or blindness

Types of vasculitis

Many forms of vasculitis can affect the brain, spinal cord, and nerves, including:

Giant cell arteritis (also known as temporal arteritis or cranial arteritis)

Giant cell arteritis affects the aorta, which brings blood from the heart to every part of the body, including the arms, legs, and head. The temporal artery (found on both sides of the head and running across the temple) and the ophthalmic artery that supplies the eyes are often affected. Giant cell arteritis typically occurs in people age 50 years and older, and is most common in people ages 70 to 89. Untreated giant cell arteritis can cause strokes and even death. Symptoms include:

• New, severe headache
• Vision problems, including blurred or double vision, or sudden vision loss
• Pain in the jaw or tongue when chewing or swallowing
• Tenderness in the temporal arteries or the scalp

Primary angiitis of the central nervous system (also known as granulomatous angiitis)

This rare disorder, which is limited to the brain and spinal cord, typically develops slowly and includes headache, dementia, behavioral changes, pain, sensory issues, and tremor. Stroke, TIA, and seizures can occur. The disorder can affect anyone of any age but peaks about age 50 and is more common in men. It is fatal if left untreated.

Takayasu's arteritis

This disease affects large arteries such as the aorta. It may cause strokes, headaches, dizziness, a feeling of cold or numbness in the limbs, problems with memory and thinking, and vision problems. It also may cause heart attacks and damage to the intestines. The disorder can cause partial to complete disability and can be fatal if left untreated. It mostly affects women between ages 20 and 40.

Polyarteritis nodosa

This rare disease can occur at any age but most often appears between ages 40 and 60 and in men more often than women. Damage to the peripheral nerves is more common than damage to the brain and spinal cord tissue. As the disease progresses, the kidneys may fail and high blood pressure may develop rapidly. Sometimes, the disease can recur after a few years. If untreated, the disorder is often fatal, ending in failure of vital organs.

Deficiency of adenosine deaminase 2 (also known as DADA2)

DADA2 is a rare, genetic form of vasculitis that is caused by a mutation in the CECR1 gene and can occur in more than one family member. Most people with DADA2 have strokes in infancy or early childhood. DADA2 was discovered by researchers at the National Institutes of Health (NIH).

Kawasaki disease

Kawasaki disease is a rare form of vasculitis that can cause stroke or brain damage in children. It primarily affects children under age 5. Kawasaki disease can cause inflammation of the walls of blood vessels in the coronary arteries, which may cause aneurysms.

Behcet's disease 

This rare disease causes chronic inflammation in the blood vessels in many parts of the body, including the brain and spinal cord. It can affect anyone at any age, but is more common in men between ages 20 and 30, particularly in the Middle East and certain Asian countries. The cause of Behcet’s disease is unknown, but may be due to genetic, immune, and environmental factors. Medication can reduce inflammation and/or help regulate the immune system. Symptoms include:

• Stroke
• Meningitis
• Cranial nerve palsies (lack of function of nerves that lead from the brain to parts of the head, face, and trunk)
• Blindness
• Memory loss
• Problems with speech, balance, and movement
• Arthritis (swollen joints)
• Cuts and scratches on the skin
• Gastrointestinal problems
• Open cuts in the mouth and on genitals
• Eye inflammation

Who is more likely to get vasculitis?

Vasculitis can affect anyone of any age. Some forms of vasculitis are more or less common based on a person’s biological sex or age. 

• Lifestyle habits such as smoking and using illegal drugs, such as cocaine, can raise a person’s risk of developing vasculitis.
• Certain medicines have been linked to a higher risk of vasculitis, such as hydralazine (used to treat high blood pressure) and levamisole (used for infections, but is also commonly added to cocaine), and tumor necrosis factor inhibitors (used to treat some autoimmune diseases).
• Other medical conditions may trigger vasculitis, including COVID-19 infection, hepatitis B or C, lymphoma, and autoimmune disorders such as lupus.

How is vasculitis in the nervous system diagnosed and treated?

Diagnosing vasculitis

Diagnosing vasculitis can be difficult, as vasculitis shares symptoms with many other disorders. Diagnosis depends on the number of blood vessels involved, their size, and their location as well as the other organs involved.

To diagnose vasculitis, a doctor will review the person's medical history, perform a physical exam to confirm signs and symptoms, and order diagnostic tests and procedures, including:

• Blood and urine tests to look for signs of inflammation (such as abnormal levels of certain proteins, antibodies, and blood cells)
• Analysis of the fluid that surrounds the brain and spinal cord (cerebrospinal fluid) to check for infection and signs of inflammation
• Biopsy of brain or nerve tissue (involving removal of a small piece of tissue that is then studied under a microscope)
• Diagnostic imaging such as CT (computed tomography)  and MRI (magnetic resonance imaging) scans to provide images of the brain, nerves, and organs, and tissues, and detect weakened or blocked vessels
• Angiogram (x-ray imaging using a special dye that is released into the bloodstream) to detect the degree of narrowing of blood vessels in the brain, head, or neck
• Ultrasound to produce high-resolution images of the blood vessel walls and organs to look for inflammation

Treating vasculitis

Treatment for vasculitis is aimed at reducing inflammation, controlling the anti-inflammatory response, and suppressing abnormal immune system activity.

Medications to treat vasculitis include:

• Corticosteroid drugs such as prednisone to reduce blood vessel inflammation
• Immunosuppressive drugs such as cyclosporine to suppress or weaken the immune system
• Monoclonal antibodies that attach to abnormally functioning immune cells (B cells) and kill them
• Blood thinners and anti-clotting medications
• Immunoglobulin therapy, which involves intravenous injections of antibodies (proteins produced by a blood cell to make a specific immune response) that are collected from a pool of healthy volunteers to lessen the infectious attack on the immune system

Other procedures to treat vasculitis include:

• Plasma exchange, or plasmapheresis, which involves taking blood out of the body and removing components in the blood's plasma that are thought to be harmful and then transfusing the blood plus replacement plasma back into the body 
• Surgery to bypass blocked vessels or treat aneurysms (although this is rare)

A doctor may also recommend healthy lifestyle changes including quitting smoking, avoiding illegal drugs, and adopting a heart-healthy lifestyle to lower the person’s risk of heart disease. 

What are the latest updates on vasculitis in the nervous system?

NINDS, a component of the National Institutes of Health (NIH), the leading supporter of biomedical research in the world, is the primary federal funding agency for research on the brain and nervous system. The National Heart, Lung, and Blood Institute (NHLBI) and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) also conduct and fund research on vasculitis.

Several NINDS-funded investigators are studying blood vessel damage and cerebral blood flow as it relates to stroke. A better understanding of the mechanisms involved in injury and repair of blood vessels in the brain may lead to improvements in the treatment and prevention of other vascular disorders, including vasculitis.

The Vasculitis Translational Research Program at NIAMS is dedicated to performing clinical and translational research in all types of vasculitis. The goal of the program is to discover factors that cause vasculitis and to develop new ways for doctors to diagnose and treat the disease.

NIH supports the Vasculitis Clinical Research Consortium, a network of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research and improving the care of individuals with various vasculitis disorders, including giant cell arteritis, polyarteritis nodosa, Takayasu's arteritis, and polyarteritis nodosa. The Consortium's website provides information about clinical research and clinical trial opportunities and helps people connect with expert doctors and patient support groups.

More information about vasculitis research supported by NINDS and other NIH institutes and centers can be found using NIH RePORTER, a searchable database of current and past research projects supported by NIH and other federal agencies. RePORTER also includes links to publications and resources from these projects.

How can I or my loved one help improve care for people with vasculitis in the nervous system?

Consider participating in a clinical trial so clinicians and scientists can learn more about vasculitis and related disorders. Clinical research with human volunteers helps researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of participants are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with vasculitis at Clinicaltrials.gov.

Learn about related topics 

• Stroke