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 comprised 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 is defined by certain patterns of distribution of blood vessel involvement, particular organ involvement, and laboratory test abnormalities. As a group, these diseases are referred to as vasculitides.

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.

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, much of which depends on what particular organs are affected.

Examples of vasculitis include:

• Kawasaki disease
• Behçet's disease
• Polyarteritis nodosa
• Granulomatosis with polyangiitis
• Cryoglobulinemia
• Takayasu's arteritis
• Churg-Strauss syndrome
• Giant cell arteritis (temporal arteritis)
• Henoch-Schönlein purpura

Vasculitis can also be accompanied by:

• Infections (such as hepatitis B)
• Exposure to chemicals (such as amphetamines and cocaine)
• Medications
• Cancers (such as lymphomas and multiple myeloma)
• Rheumatic diseases (such as rheumatoid arthritis and systemic lupus erythematosus)

Symptoms of vasculitis vary greatly from person to person and depend on the affected organs and the severity of the condition.

Generally, vasculitis causes fatigue and malaise. Sometimes vasculitis can lead to weakness and weight loss.

• Skin: Rashes, skin discoloration, and ulcers
• Muscles: Muscle pain
• Lungs: Shortness of breath and cough
• Heart: Congestive heart failure
• Brain: Headaches, confusion, seizures, stroke, paralysis, numbness, and lightheadedness
• Kidneys: Kidney failure

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, lungs, nerves, and kidneys. 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 in affected blood vessels.

The treatment of the various forms of vasculitis is based on the severity of the illness and the organs involved.

• Treatments are generally directed toward stopping 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.