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NINDS Vasculitis Syndromes of the Central and Peripheral Nervous Systems Information Page

Synonym(s):   Temporal Arteritis, Cranial Arteritis, Giant Cell Arteritis
Condensed from Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet

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What is Vasculitis Syndromes of the Central and Peripheral Nervous Systems ?

Vasculitis is an inflammation of blood vessels, which includes the veins, arteries, and capillaries.  Researchers think that inflammation occurs with infection or is thought to be due to a faulty immune system response. Vasculitic disorders can cause problems in any organ system, including the central (CNS) and peripheral (PNS) nervous systems.  Vasculitis 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.    

A vasculitic syndrome may begin suddenly or develop over time.  Symptoms include:  headaches, especially a headache that doesn’t go away; fever; 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; and visual disturbances, such as double vision, blurred vision, or blindness

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), Primary angiitis of the CNS (granulomatous angiitis), Takayasu’s disease, Periarteritis nodosa, Kawasaki disease, Churg-Strauss syndrome, Wegener’s granulomatosis, systemic lupus erythematosis, scleroderma, rheumatoid arthritis, Sjogren’s syndrome, and Behcet’s disease.

Is there any treatment?

Treatment for a vasculitis syndrome depends upon the specific diagnosis.  Most of the syndromes respond well to steroid drugs, such as prednisolone.  Some may also require treatment with an immunosuppressive drug, such as cyclophosphamide.

What is the prognosis?

The prognosis is dependent upon the specific syndrome, however, most of the syndromes are fatal if left untreated.

What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health (NIH) conduct research relating to vasculitis syndromes in laboratories at the NIH and also support vasculitis 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.

NIH Patient Recruitment for Vasculitis Syndromes of the Central and Peripheral Nervous Systems Clinical Trials

Organizations

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American Autoimmune Related Diseases Association
22100 Gratiot Avenue
Eastpointe, MI   48021-2227
aarda@aarda.org
http://www.aarda.org
Tel: 586-776-3900 800-598-4668
Fax: 586-776-3903

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT   06810
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

National Eye Institute (NEI)
National Institutes of Health, DHHS
31 Center Drive, Rm. 6A32 MSC 2510
Bethesda, MD   20892-2510
2020@nei.nih.gov
http://www.nei.nih.gov
Tel: 301-496-5248

National Institute of Allergy and Infectious Diseases (NIAID)
National Institutes of Health, DHHS
6610 Rockledge Drive, MSC 6612
Bethesda, MD   20892-6612
http://www.niaid.nih.gov
Tel: 301-496-5717

Related NINDS Publications and Information


Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

Last updated April 16, 2014