Vasculitis FS (E).indd 1 

U.S. DEPARTMENT OF HEALT

AND HUMAN SERVICES

Public Health Service

National Institutes of Health

Vasculitis Syndromes 

of the Central and Periphera

Nervous Systems 
Vasculitis FS (E).indd 2 
Vasculitis FS (E).indd 1 

Vasculitis Syndromes of 

the Central and Periphera

Nervous Systems 

What is vasculitis? 

Vasculitis is an inflammation of blood vessels, which includes the veins, arterie

and capillaries. Depending on the type, 

vasculitis can affect blood vessels of any type

size, or location. Inflammation occurs with 

infection or is thought to be due to a faulty 

immune system response. Dysfunction may 

occur due to the inflammation itself or over 

time as the blood vessel walls swell, harden, 

thicken, and develop scar tissue. This narrow

the passage through which blood can flow. 

As the condition progresses, it can slow or 

completely stop the normal flow of blood. 

How does vasculitis affect the 

nervous system? 

Vasculitis can cause problems in any orgasystem, including the central (CNS) and 

peripheral (PNS) nervous systems. Vasculitic 

disorders, or syndromes, of the CNS and PNS 

are characterized by the presence of inflammatory cells in and around blood vessels, 

and secondary narrowing or blockage of the 

blood vessels that nourish the brain, spinal 

cord, or peripheral nerves. Any type or size 

of blood vessel may be involved—arteries, 

arterioles, veins, venules, or capillaries. 
Vasculitis FS (E).indd 2 

What are the symptoms? 

Avasculitis syndrome may begin suddenlor develop over time. Symptoms include

•	 headaches, especially a headache that 

doesn’t go away 

•	 fever 

•	 malaise (feeling out-of-sorts) 

•	 rapid weight loss 

•	 confusion or forgetfulness leading to 

dementia 

•	 aches and pains in the joints and muscles 

•	 pain while chewing or swallowing 

•	 paralysis or numbness, usually in the 

arms or legs 

•	 visual disturbances, such as double vision, 

blurred vision, or blindness 

•	 seizures, convulsions 

•	 stroke or transient ischemic attack (TIA, 

sometimes also called a “mini-stroke”) 

•	 unusual rashes or skin discoloration 

•	 problems with the kidneys or other organs 

How are these syndromes diagnosed? 

Adoctor who suspects CNS or PNS vasculitis will gather a comprehensive 

medical history of the individual, perform 

a physical examination, order laboratory 

tests (primarily blood tests), and recommend 

any other tests that seem appropriate. Electromyography and nerve conduction studies 

identify blocks and loss of nerve supply to 

muscle due to vasculitic nerve damage. 

2
Vasculitis FS (E).indd 3 

Diagnostic imaging of the brain blood vessels 

such as magnetic resonance or computed 

tomography angiograms can sometimes 

identify narrowing in the larger blood vessel

Direct injection of a contrast dye into brain 

blood vessels may be needed to look for 

narrowings consistent with vasculitis in 

medium-sized brain arteries. 

However, the diagnosis of vasculitis often 

requires evidence that there is ongoing 

inflammation. Inflammatory cells may be 

found in the spinal fluid. Often there is a need 

to conduct a tissue biopsy to examine blood 

vessels under a microscope. In some cases a 

brain biopsy may be necessary to evaluate th

compromised tissue. A definitive diagnosis 

is important because the treatment usually 

requires powerful immune-suppressive drug

In addition, it is important to make sure that 

an infection is not causing the inflammation. 

What are some of these syndromes called

T he diagnosis of a CNS or PNS vasculitis disorder will depend upon the number of 

blood vessels involved, their size, and their 

location in the CNS or PNS as well as the 

types of organs involved. Although these 

disorders are rare, there are many of them. 

Some of the better understood syndromes are

Temporal arteritis (also called giant cell 

arteritis or cranial arteritis) 

Temporal arteritis is a common chronic 

inflammatory disease of large blood vessels 

occurring primarily in people 50 and older. I

most often involves narrowing and sometime

blockage of the arteries that bring blood to 
Vasculitis FS (E).indd 4 

the brain. Doctors will diagnose temporal 

arteritis if at least three of the following 

symptoms are present: 

•	 new, severe headache 

•	 visual disturbances 

•	 pain in the jaw or tongue when chewing 

or swallowing 

•	 tenderness in the temporal arteries 

(the arteries that run across the temples 

on either side of the head) or the scalp 

Fever, weight loss, and neck or muscle pain 

can occur, usually in the early phase of the 

disease. Individuals may also have arthritis; 

carpal tunnel syndrome; fatigue; and weakness, paralysis, or numbness in isolated 

muscles. The disease is usually limited to 

one to two years and is rarely fatal. 

Abrupt but reversible blindness is the most 

dramatic complication of temporal arteritis. 

About one in ten individuals with temporal 

arteritis will develop blindness in one eye, 

preceded by visual disturbances. Once one 

eye is affected, three out of four individuals 

will go on to lose vision in the other eye, 

most in two weeks or less. 

The main goal of treatment for temporal 

arteritis is to prevent blindness. Most individuals respond well to steroid drugs, such 

as prednisone and methylprednisolone, but 

they must be given promptly and carefully 

monitored. Long-term use of steroids can 

cause harmful side effects, such as collapsing 

vertebrae, muscle pain, diabetes, cataracts, 

and infection. 

4
Vasculitis FS (E).indd 5 

Primary angiitis of the CNS 

(granulomatous angiitis) 

The symptoms of this rare disorder develop 

slowly. Symptoms include headache and 

encephalopathy-like symptoms such as dementi

and tremor. Stroke, TIA, and seizures can occu

Definitive diagnosis may require brain biops

Treatment includes steroid and immunosuppressive drugs, such as prednisolone and 

cyclophosphamide. It is fatal if left untreated

Takayasu’s disease 

This disease affects large arteries such as 

the aorta, which brings blood to the arms, 

legs, and head. It primarily strikes individuals 

of Asian descent and predominantly affects 

females under the age of 40. The main symptoms are fainting and visual disturbances 

and it may also cause stroke. Although the 

disorder is serious, the prognosis is positive: 

more than 90 percent of those diagnosed 

with Takayasu’s disease survive beyond a 

decade after diagnosis. Steroid drugs are 

used in the early phase of the disease, but 

some individuals become steroid-resistant 

and have to switch to cyclophosphamide 

or low-dose methotrexate. 

Periarteritis nodosa 

The onset of this rare and serious disease is 

generally between the ages of 40 and 50, but 

it can occur at any age. Men are three times 

more likely to develop the disease than women

Symptoms can mimic those of many other 

diseases, but the most common initial complaints are fever, abdominal pain, numbness 

or pain in the legs and limbs, weakness, and 
Vasculitis FS (E).indd 6 

unexplained weight loss. As the disease progresses, the kidneys may fail and high blood 

pressure may develop rapidly. Certain drugs 

(for example, those in the sulfa family), vaccines, bacterial infections, and viral infection

have been associated with the onset of the 

disease. Damage to the PNS with neuropathy 

is more common than damage to the CNS, bu

if the disease does involve the CNS, damage 

to brain and spinal cord tissue can occur. 

The disease is treated aggressively with high 

doses of steroids and immunosuppressive drug

such as cyclophosphamide. Eighty percent 

of individuals who receive appropriate treatment are alive five years later. Untreated 

disease is often fatal, ending in heart failure

kidney failure, or failure of other vital organ

Are there additional vasculitis disorders 

that can cause neurological symptoms? 

Other vasculitis syndromes include Kawasaki disease, which can cause 

stroke or encephalopathy in children; ChurgStrauss syndrome; Wegener’s granulomatosis

systemic lupus erythematosis; scleroderma; 

rheumatoid arthritis; Sjogren’s syndrome; 

and Behcet’s disease. 

What research is being done to better 

understand these syndromes? 

T he National Institute of Neurological Disorders and Stroke (NINDS), a component 

of the National Institutes of Health (NIH), 

and other NIH institutes conduct research 

relating to vasculitis syndromes in laboratories at the NIH and also support vasculitis 

6
Vasculitis FS (E).indd 7 

research through grants to major medical 

institutions across the country. 

The NINDS supports The Vasculitis Clinical 

Research Consortium (VCRC), 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 

vasculitis, including Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss 

syndrome, polyarteritis nodosa, Takayasu’s 

arteritis, and temporal arteritis. The medica

centers are located at Boston University Schoo

of Medicine, Cleveland Clinic Foundation, 

The Johns Hopkins Vasculitis Center, and 

Mayo Clinic College of Medicine. The Consortium’s internet site provides information 

about clinical research and clinical trial 

opportunities and helps individuals connect 

with expert doctors and patient support group

Where can I get more information? 

For more information on neurological disorders or research programs funded 

by the National Institute of Neurological 

Disorders and Stroke, contact the Institute’s 

Brain Resources and Information Network 

(BRAIN) at: 

BRAIN 

P.O. Box 5801 

Bethesda, MD 20824 

800-352-9424 

www.ninds.nih.gov
Vasculitis FS (E).indd 8 

Information also is available from the 

following organizations: 

American Autoimmune Related 

Diseases Association 

22100 Gratiot Avenue 

East Detroit, MI 48021-2227 

586-776-3900 

800-598-4668 

www.aarda.org

National Organization for Rare 

Disorders (NORD) 

P.O. Box 1968 

(55 Kenosia Avenue) 

Danbury, CT 06813-1968 

203-744-0100 Voice Mail 

800-999-NORD (6673) 

www.rarediseases.org

National Eye Institute (NEI) 

National Institutes of Health, DHHS 

31 Center Drive, Rm. 6A32 MSC 2510 

Bethesda, MD 20892-2510 

301-496-5248 

www.nei.nih.gov

National Institute of Allergy and Infectious 

Diseases (NIAID) 

National Institutes of Health, DHHS 

6610 Rockledge Drive, MSC 6612 

Bethesda, MD 20892-6612 

301-496-5717 

www.niaid.nih.gov

8
Vasculitis FS (E).indd 9 
Vasculitis FS (E).indd 10 

Prepared by: 

Office of Communications and Public Liaison 

National Institute of Neurological 

Disorders and Stroke 

National Institutes of Health 

Department of Health and Human Services 

Bethesda, Maryland 20892 -2540 

NIH Publication No. 11 -5596 July 2011