Vasculitis

Inflammation of the blood vessels (vasculitis) may be caused when antibodies that have attached to antigens in the blood (immune complexes), attach to the blood vessel walls. These purplish spots can be felt in the skin. They do not turn white (blanch) when pressed. As the condition progresses, they may become larger and more bruise-like (ecchymotic), and some may develop central ulceration or necrosis (tissue death).

Information about Vasculitis

Vasculitis means that blood vessels are inflamed. When part of your body is inflamed, it swells and is usually uncomfortable or painful (although with many types of vasculitis you will not be able to see any swelling on the outside of the body). The term '-itis' means inflammation, so, for example, when you have appendicitis, your appendix is inflamed; and when you have arthritis, your body joints are inflamed.

Blood vessels are the tubes which carry blood around your body. There are three types of blood vessel which can be affected by vasculitis:

• arteries, which take blood from the heart to various parts of the body – to body organs (like the kidney and liver) and to body tissues (like your skin)
• veins, which take blood back to the heart
• capillaries, which are tiny vessels between the arteries and the veins where oxygen and other materials pass from the blood into your tissues. ).

Our body organs and tissues need a regular blood supply to work properly. If the blood vessels are inflamed, this can block or reduce the flow of blood. The wall of the blood vessel can also bulge – this is what doctors call an aneurysm. An aneurysm can burst (rupture) and cause problems. The diagram below shows an inflamed blood vessel where the blood flow has been reduced.

The damage which vasculitis can cause depends upon which part of the body is affected – the bigger the blood vessel the greater the potential damage. And the more important the body tissue supplied, the more serious the damage will be. For example, damage to the small blood vessels in the skin will cause a rash. Small ‘spots’ of dead skin will form but these will heal over once the vasculitis settles. This is not serious. On the other hand, if the blood vessels which go to the kidney are damaged, this could stop your kidney working altogether. Urgent treatment is then needed, and if both kidneys stop working, regular dialysis would be needed to do the job normally done by the kidneys.

Vasculitis can appear suddenly in someone who has previously been completely well – doctors call this primary vasculitis. Vasculitis can also appear in people who have an established disease such as arthritis – this is called secondary vasculitis. It may happen, for example, with rheumatoid arthritis, systemic lupus erythematosus (SLE) or Sjögren’s syndrome (see the separate arc booklets for more information on these conditions: Rheumatoid Arthritis, Lupus and Sjögren’s Syndrome).

Doctors usually define the types of vasculitis according to the size of the blood vessels involved. All these different types are explained later in this booklet:

• Vasculitis in large arteries This includes temporal arteritis and Takayasu arteritis.
• Vasculitis in medium-sized arteries This includes polyarteritis nodosa and Kawasaki disease. Medium-sized arteries can also be involved in vasculitis which occurs with (is secondary to) rheumatoid arthritis, systemic lupus erythematosus and Sjögren’s syndrome.
• Vasculitis in small arteries This includes Wegener's granulomatosis, microscopic polyangiitis and Churg–Strauss syndrome. Vasculitis in the small arteries can also be a consequence of rheumatic diseases, including rheumatoid arthritis and systemic lupus erythematosus. It can happen with infections including hepatitis and, very occasionally, with different types of cancers, including leukaemia and lymphomas.
• Vasculitis in small vessels (usually capillaries) This usually involves the skin and is also sometimes caused by a reaction to some drugs.

Symptoms of Vasculitis

Some types of vasculitis appear to be due to a type of allergic response to a specific substance (for example, a drug). Other types of vasculitis have no identifiable initiating event. Furthermore, researchers have not been able to consistently identify antibody-antigen complexes in all of the types of diseases caused by vasculitis. The types of antigens responsible for the initial immune response have often gone unidentified as well. Furthermore, not all people with such complexes deposited along the blood vessels go on to develop vasculitis. Some researchers believe that, in addition to the presence of immune complexes, an individual must have some other characteristics which make him or her susceptible to vasculitis. Many questions have yet to be answered to totally explain the development these diseases.

Symptoms

Symptoms of vasculitis depend on the severity of the inflammation and the organ system or systems affected. Some types of vasculitis are so mild that the only symptoms noted are small reddish-purple dots (called petechiae) on the skin due to tiny amounts of blood seeping out of leaky blood vessels. In more widespread types of vasculitis, the patient may have general symptoms of illness, including fever, achy muscles and joints, decreased appetite, weight loss, and loss of energy. The organ systems affected by vasculitis may include:

• The skin. Rashes, bumps under the skin, petechiae, larger reddish-purple circles (purpura), or bruising (ecchymoses) may appear. Areas of skin totally deprived of blood flow, and therefore of oxygen, may die, resulting in blackened areas of gangrene.
• The joints. In addition to joint pain, the joints themselves may become inflamed, resulting in arthritis.
• Brain and nervous system. Inflammation of the blood vessels in the brain can cause headaches, changes in personality, confusion, and seizures. If an area of the brain becomes totally deprived of oxygen, a stroke occurs. A stroke means that an area of brain tissue is either severely injured or completely dead from lack of oxygen. This may leave the individual with a permanent disability. If the vessels that lead to the eyes are affected, vision may become seriously disturbed. Nerves in the arms and legs may result in painful tingling sensations, loss of feeling, and weakness.
• Gastrointestinal system. Patients may have significant abdominal pain, vomiting, and diarrhea. If blood flow is completely cut off to an area of intestine, that part of the intestine will die off. The liver may be affected.
• Heart. This is an extremely serious type of vasculitis. The arteries of the heart (coronary arteries) may develop weakened areas, called aneurysms. The heart muscle itself may become inflamed and enlarged. With oxygen deprivation of the heart muscle, the individual may suffer a heart attack.
• Lungs. The patient may experience shortness of breath with chest pain, and may cough up blood. There may be wheezing.
• Kidney. Changes in the arteries of the kidney may result in high blood pressure. The kidneys may become increasingly unable to appropriately filter the blood, and kidney failure may occur.

Specific diseases associated with Vasculitis

Multiple types of disease are associated with vasculitis. Many autoimmune diseases have vasculitis as one of their complications. These include systemic lupus erythematosus, rheumatoid arthritis, scleroderma, and polymyositis. Other types of diseases which have vasculitis as their major manifestations include:

• Polyarteritis nodosa. This is an extremely serious, systemic (affecting systems throughout the body) form of vasculitis. Small and medium arteries are involved, and the inflammation is so severe that the walls of the arteries may be destroyed. Any organ system, or multiple organ systems, may be affected. The most serious effects include kidney failure, complications involving the heart, gastrointestinal problems, and high blood pressure.
• Kawasaki's disease is an acute disease which primarily strikes young children. Fever and skin manifestations occur in all patients. While most patients recover completely, a few patients suffer from vasculitis in the heart. This is frequently fatal.
• Henoch-Schonlein purpura. While this frequently occurs in children, adults may also be affected. This disease tends to affect the skin, joints, gastrointestinal tract, and kidneys.
• Serum sickness occurs when an individual reacts to a component of a drug, for example penicillin. Symptoms of this are often confined to the skin, although fevers, joint pain, and swelling of lymph nodes may also occur.
• Temporal arteritis (also called giant cell arteritis) tends to involve arteries which branch off the major artery that leads to the head, called the carotid. An artery which feeds tissues in the area of the temple (the temporal artery) is often affected. Severe headaches are the most classic symptom. Other symptoms include fatigue, loss of appetite and then weight, fever, heavy sweating, joint pain, and pain in the muscles of the neck, shoulders, and back. If the vasculitis includes arteries which supply the eye, serious visual disturbance or even blindness may result.
• Takayasu's arteritis affects the aorta (the very large main artery that exits the heart and receives all of the blood to be delivered throughout the body), and arteries which branch off of the aorta. Initial symptoms include fatigue, fever, sweating at night, joint pain, and loss of appetite and weight. Every organ may be affected by this disease. A common sign of this disease is the inability to feel the pulse in any of the usual locations (the pulse is the regular, rhythmic sensation one can feel with a finger over an artery, for example in the wrist, which represents the beating of the heart and the regular flow of blood).
• Wegener's granulomatosis: This disease exerts its most serious effects on the respiratory tract. The vasculitis produced by this disease includes the formation of fibrous, scarring nodules called granulomas. Symptoms include nose bleeds, ear infections, cough, shortness of breath, and chest pain. There may be bleeding in the lungs, and a patient may cough up blood. The kidneys, eyes, and skin are also frequently involved.

Diagnosis of Vasculitis

Diagnosis of any type of vasculitis involves demonstrating the presence of a strong inflammatory process. Tests which reveal inflammation throughout the body include erythrocyte sedimentation rate, blood tests which may reveal anemia and increased white blood cells, and tests to demonstrate the presence of immune complexes and/or antibodies circulating in the blood. An x-ray procedure, called angiography, involves injecting dye into a major artery, and then taking x-ray pictures to examine the blood vessels, in order to demonstrate the presence of inflammation of the vessel walls. Tissue samples (biopsies) may be taken from affected organs to demonstrate inflammation.

Treatment of Vasculitis

Even though there are many different types of vasculitis, with many different symptoms based on the organ system affected, treatments are essentially the same. They all involve trying to decrease the activity of the immune system. Steroid medications (like prednisone) are usually the first types of drugs used. Steroids work by interfering with the chemicals involved in the inflammatory process. More potent drugs for severe cases of vasculitis have more serious side effects. These include drugs like cyclophosphamide. Cyclophosphamide works by actually killing cells of the patient's immune system.

Prognosis of Vasculitis

The prognosis for vasculitis is quite variable. Some mild forms of vasculitis, such as those brought on by reactions to medications, may resolve totally on their own and not even require treatment. Temporal arteritis, serum sickness, Henoch-Schonlein purpura, and Kawasaki's disease usually have excellent prognoses, although when Kawasaki's affects the heart, there is a high death rate. Other types of vasculitis were always fatal, prior to the availability of prednisone and cyclophosphamide, and continue to have high rates of fatal complications. These include polyarteritis nodosa and Wegener's granulomatosis.