Vasculitis Syndromes of the Central and Peripheral Nervous Systems

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 systemresponse. 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.

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.

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