Vasculitis Syndrome Vasculitis Syndrome
National Institute of Neurological Disease and Stroke

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.