What is Vasculitis?

 

The brain and arteries at base of the brain
Grays Anatomy

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
• 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 prednisone.  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

• At NIH Clinical Center
• Throughout the U.S. and Worldwide
• NINDS Clinical Trials

Organizations

Column1	Column2
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

• Vasculitis Fact Sheet brochure ( pdf file, 426 kb)
                               
  Prepared by:
  Office of Communications and Public Liaison
  National Institute of Neurological Disorders and Stroke
  National Institutes of Health
  Bethesda, MD 20892     

All NINDS-prepared information is in the public domain and may be freely copied.   

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