Vasculitic Neuropathy

Definition

Vasculitic neuropathy refers to damage to the peripheral nerves (the nerves that are located outside of the brain and spinal cord) as a consequence of vasculitis (a condition characterized by inflammation and destruction of blood vessels).

Description

Vasculitis refers to a number of conditions that cause inflammation in the blood vessels of the body. This inflammation can prevent sufficient blood flow from reaching various organs and tissues of the body. Because adequate blood flow is required to provide the organs and tissues with oxygen, vasculitis causes damage to oxygen-deprived organs and tissues. When peripheral nerves are oxygen-deprived due to vasculitis, vasculitic neuropathy ensues.

Peripheral neuropathy can occur as the only symptom of vasculitis, or it can be part of a symptom complex.

Demographics

About 60-70% of all patients with vasculitis will experience peripheral neuropathy. In fact, about 34% of all patients with vasculitis will manifest peripheral neuropathy as the sole manifestation of their vasuclitis. The average age of an individual with vasculitic neuropathy is 62.

Causes and symptoms

Vasculitic neuropathy can accompany a number of types of vasculitis, including polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, Sjogren's syndrome, rheumatoid vasculitis, and vasculitis due to infections (such as Lyme disease, hepatitis, or HIV). Vasculitis occurs when the body's immune system accidentally misidentifies markers on the blood vessel walls as foreign. The immune system then begins to produce immune cells that attack and destroy the blood vessels. As the blood vessels become inflamed, blood flow through them is diminished, resulting in oxygen deprivation of the organs or tissues they normally serve. When the oxygen-deprived tissues are nerve cells, vasculitic neuropathy results.

Most people with vasculitic neuropathy notice pain and then weakness in a random, nonsymmetric distribution throughout their limbs; a smaller number (about one-third of all sufferers) notice pain and weakness that progress in a symmetric fashion, beginning with the feet or hands and progressing up the limbs. The pain of vasculitic neuropathy can include shooting, sharp pain, tingling, numbness, burning, and stinging. Some patients with vasculitic neuropathy will also experience fever, decreased appetite, weight loss, rash, fatigue, joint pain, and kidney problems.

Diagnosis

Examining a sample of an affected nerve cell (biopsy) will allow the diagnosis to be made. The biopsy will demonstrate the inflammation and destruction of blood vessel walls characteristic of vasculitis. Electrodiagnostic studies use needle electrodes to stimulate affected nerves or muscles, in order to demonstrate a slow or abnormal response.

Treatment team

Vasculitic neuropathy may be treated by a neurologist or a rheumatologist. Physical and occupational therapists can help optimize recovery of function.

Treatment

Treatment for vasculitic neuropathy involves medications that decrease inflammation and suppress the activity of the immune system. Such medications include cortciosteroids and cyclophosphamide. Physical and occupational therapy can help restore functioning and can provide strategies to help overcome any permanent disabilities caused by the vasculitic neuropathy.

Prognosis

Once treatment for vasculitic neuropathy has been initiated, symptom progression should halt, and the condition should stabilize. Some improvement in already established symptoms is possible; pain may decrease, and some degree of weakness may improve, although recovery of function is usually very slow and only partial.

BOOKS

Chalk, Colin. "Peripheral Neuropathies." In Conn's Current Therapy 2004, 56th ed., edited by Shaun Ruddy, et al. Elsevier, 2004.

Griffin, John W. "Immune-Mediated Neuropathies." In Cecil Textbook of Internal Medicine, edited by Lee Goldman, et al. Philadelphia: W. B. Saunders Company, 2000.

PERIODICALS

Griffin, John W. "Vasculitic Neuropathies." Rheumatic Diseases Clinics of North America 27 (November 2001): 751–760.

Nadeau, S. E. "Neurologic Manifestations of Systemic Vasculitis." Neurologic Clinics 20, no. 1 (February 2002): 123–150.

Pascuzzi, Robert M. "Peripheral Neuropathies in Clinical Practice." Medical Clinics of North America 87 (May 2003): 697–724.

Rosalyn Carson-DeWitt, MD