Vasculitis Syndromes

What are vasculitis syndromes?

Vasculitis is inflammation of blood vessels, which includes the veins, arteries, and capillaries. It occurs when the immune system mistakenly attacks a blood vessel.

Vasculitis can also be caused by other immune system disease, an allergic reaction to medicines or toxins, and by certain blood cancers that trigger an immune system reaction. The resulting reduced blood flow can permanently damage the brain, spinal cord, peripheral nervous system, and other organs and tissue.

Some forms of vasculitis affect a particular organ, while others may affect many organs at the same time.

Symptoms include headaches (especially a headache that doesn’t go away), fever, weight loss, confusion or forgetfulness leading to dementia, swelling of the brain, pain, vision problems, trouble speaking or understanding, muscle weakness and paralysis, and seizures.

Some of the better understood vasculitis syndromes are temporal arteritis (also called giant cell arteritis or cranial arteritis—a chronic inflammatory disorder of large blood vessels) and Takayasu’s disease, which affects larger aortas and may cause stoke.

Is there any treatment?

Treatment for a vasculitis syndrome depends upon the specific diagnosis (which can be difficult, as some diseases have similar symptoms of vasculitis) and the organ(s) affected.

Medications that may suppress the abnormal immune system activity include glucocorticoid drugs such as prednisone, immunosuppressive drugs such as cyclophosphamide, and rituximab. Long-term treatment is usually needed.

Aneurysms involved with vasculitis may be treated surgically.

What is the prognosis?

The prognosis is dependent upon the specific syndrome. Some forms of vasculitis can cause stroke, while others affect other organs as well.  Some of the syndromes are fatal if left untreated.

What research is being done?

Several investigators funded by the National Institute of Neurological Disorders and Stroke (NINDS) are studying blood vessel damage and cerebral blood flow as it relates to stroke.

The NINDS also funds research on vascular cognitive impairment, which is an important contributor to aging-related cognitive decline and is the result of impaired performance of the brain's small blood vessels.

Additionally, the NINDS and other institutes of the National Institutes of Health (NIH) support 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 various vasculitis disorders.

Also, the Vasculitis Translational Research Program at NIH's National Institute of Arthritis and Musculoskeletal and Skin Diseases performs clinical and translational research on all types of vasculitis.