Vasculitis syndromes of the central and peripheral nervous systems

Vasculitis is inflammation of blood vessels, which includes the veins, arteries, and capillaries.

Vasculitis

Pathophysiology

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.

Clinical features

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

Symptoms

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 stroke.

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.

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 activitiy 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.