Vasculitis
(Angiitis)

Medical Author: William C. Shiel Jr., MD, FACP, FACR

• What is vasculitis?
• What causes vasculitis and what are examples of diseases with vasculitis?
• How is vasculitis diagnosed?
• How is vasculitis treated?

What is vasculitis?

Vasculitis is a general term for a group of uncommon diseases that feature inflammation of the blood vessels. The blood vessels of the body are referred to as the vascular system. The blood vessels are composed of arteries that pass oxygen-rich blood to the tissues of the body and veins that return oxygen-depleted blood from the tissues to the lungs for oxygen. Vasculitis is characterized by inflammation in and damage to the walls of various blood vessels.

Each of the vasculitis diseases (also, as a group, referred to as vasculitides) is defined by certain patterns of distribution of blood vessel involvement, particular organ involvement, and laboratory test abnormalities.

The word vasculitis is derived from the Latin "vasculum", vessel + "- itis", inflammation. Another term for vasculitis is angiitis. When arteries are the inflamed blood vessels, the condition is also referred to as arteritis. When the veins are inflamed, it is referred to as venulitis.

What causes vasculitis and what are examples of diseases with vasculitis?

The actual cause of these vasculitis diseases is usually not known. However, immune system abnormality and inflammation of blood vessels are common features. Each form of vasculitis has its own characteristic pattern of symptoms. Examples of vasculitis include Kawasaki disease, Behcet's disease, polyarteritis nodosa, Wegener's Granulomatosis, Takayasu's Arteritis, Churg-Strauss Syndrome, giant cell arteritis (temporal arteritis), and Henoch Schonlein Purpura.

Vasculitis can also accompany infections (such as hepatitis B), exposure to chemicals (such as amphetamines and cocaine), medications, cancers (such as lymphomas and multiple myeloma), and rheumatic diseases (such as rheumatoid arthritis, systemic lupus erythematosus).

How is vasculitis diagnosed?

Laboratory testing of blood or body fluids in a patient with active vasculitis generally indicates inflammation in the body. Depending on the degree of organ involvement, a variety of organ function tests can be abnormal.

The diagnosis of vasculitis is definitively established after a biopsy of involved tissue demonstrates the pattern of blood vessel inflammation. Examples of tissues used for biopsy include skin, sinuses, lung, nerve, and kidney. Depending upon the situation, an alternative to biopsy can be an x-ray test of the blood vessels called an angiogram, which can demonstrate characteristic patterns of inflammation.

How is vasculitis treated?

The treatment of the various forms of vasculitis depends on the severity of the illness and the organs involved. Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, cortisone-related medications, such as prednisone, are used. Additionally, other immune suppression drugs, such as cyclophosphamide (Cytoxan) and others, are considered. Additionally, affected organs (such as the heart or lungs) may require specific medical treatment when the disease is active.

The management of vasculitis is an evolving field in medicine. The ideal programs for monitoring and treatment will continue to improve as disease patterns and causes are defined by medical research.

Vasculitis At A Glance

• Vasculitis is a group of uncommon diseases which feature inflammation of the blood vessels.
• Diagnosis of vasculitis can be confirmed by a biopsy of involved tissue or angiography.
• Treatment is directed toward decreasing the inflammation of the arteries and improving the function of affected organs.

For further information, the Medical Editors recommend reviewing specific vasculitis diseases in the database.

Reference:
Clinical Primer of Rheumatology, Lippincott Williams & Wilkens, edited by William Koopman, et. al., 2003.
Kelley's Textbook of Rheumatology, W B Saunders Co, edited by Shaun Ruddy, et.al., 2000.