Vasculitis is an inflammation of the vascular system, which includes the veins, arteries, and capillaries. Researchers think that inflammation is due to a faulty immune system response. Vasculitis 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 vasculitis 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.
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.
The prognosis is dependent upon the specific syndrome, however, most of the syndromes are fatal if left untreated.
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.
American Autoimmune Related Diseases Association 22100 Gratiot Avenue Eastpointe, MI 48201-2227 aarda@aarda.org http://www.aarda.org Tel: 586-776-3900 800-598-4668 Fax: 586-776-3903 |
National Organization for Rare Disorders (NORD) P.O. Box 1968 (55 Kenosia Avenue) Danbury, CT 06813-1968 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 |
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 February 20, 2008