Vasculitis

Vasculitis is an inflammation of the blood vessels in the body. In vasculitis, the body’s immune system mistakenly attacks the body’s own blood vessels, causing them to become inflamed. Inflammation can damage the blood vessels and lead to a number of serious complications.

Vasculitis can affect any of the body’s blood vessels. These include arteries, veins, and capillaries. Arteries are vessels that carry blood from the heart to the body’s organs, veins are the vessels that carry blood back to the heart, and capillaries are the tiny blood vessels that connect the small arteries and veins.

When a blood vessel becomes inflamed, it can:

• Narrow, making it more difficult for blood to get through
• Close off completely so that blood can’t get through at all (occlusion)
• In rare cases, stretch and weaken so much that it bulges (aneurysm) and may possibly burst (aneurysm rupture)

Figure A shows a normal artery with normal blood flow (the inset image shows a cross-section of the normal artery). Figure B shows an artery narrowed due to inflammation in the arterial wall, causing decreased blood flow (the inset image shows a cross-section of the inflamed artery). Figure C shows a totally occluded (blocked) artery due to inflammation and scarring in the arterial wall (the inset image shows a cross-section of the block artery). Figure D shows an artery containing an aneurysm. [Credit: NIH]

Types

There are many different disorders that are classified as types of vasculitis. Although all of these disorders include inflammation of the blood vessels, most differ from each other in terms of what populations of people and which organs they affect.

Behcet’s Disease

Behcet’s disease can affect any part of the body because it can occur in vessels of any size or type. Commonly affected organs include the eyes, the mouth, and the genitals. Patients with this type of vasculitis are often younger than those with other types, many times in their twenties and thirties. The HLA-B51 gene allele is often associated with Behcet’s disease, but not everyone with the gene will develop the disease. Behcet’s is more common in individuals of Mediterranean, Middle Eastern, or Far Eastern descent, but is usually not found in Blacks.

Buerger’s Disease

Buerger’s disease typically affects blood flow to the hands and feet. Buerger’s disease is also known as thromboangiitis obliterans and is more likely to occur in smokers than nonsmokers.

Central Nervous System Vasculitis

Central nervous system vasculitis affects the brain and sometimes the spinal cord.

Churg-Strauss Syndrome

Churg-Strauss syndrome can affect many different organs, but it most commonly affects the lungs, skin, kidneys, and heart. Many people who are diagnosed with Churg-Strauss syndrome also have asthma. They may have had preexisting, newly diagnosed, or recently worsened asthma when diagnosed with Churg-Strauss syndrome.

Cryoglobulinemia

Common signs and symptoms of cryoglobulinemia are a rash on the lower limbs, arthritis, and nerve damage. Type 1 cryoglobulinemia is often associated with lymphoma, while Type 2 cryoglobulinemia is often associated with hepatitis C infection.

Giant Cell Arteritis

Common symptoms of giant cell arteritis include headache, fever, blurred vision, and pain in the jaw, shoulders, or hips. It is the most common form of vasculitis that occurs in adults older than 50 years. It is more likely to occur in people of Scandinavian origin, but can occur in people of any race.

Henoch-Schonlein Purpura

Henoch-Schonlein purpura most commonly affects the skin, kidneys, joints, and stomach. It is the most common form of vasculitis found in children, but can occur at any age. When seen in children, it often follows an upper respiratory infection. It is not usually a chronic disease, and full recovery is common.

Microscopic Polyangiitis

Microscopic polyangiitis most commonly affects the kidneys, skin, and nerves, but it can affect any organ in the body.

Polyarteritis Nodosa

Polyarteritis nodosa most commonly affects the kidneys, skin, and nerves, but it can affect any organ in the body. It is most common in people in their thirties and forties. Men are twice as likely as women to get polyarteritis nodosa. In some cases, it is associated with chronic hepatitis B infection or a very specific type of leukemia known as hairy cell leukemia.

Polymyalgia Rheumatica

Polymyalgia rheumatica most commonly affects the large joints in the body, such as the shoulders and hips. It often occurs along with giant cell arteritis.

Rheumatoid Vasculitis

Rheumatoid vasculitis can affect many different organs, including the eyes, skin, hands, and feet. It commonly occurs in patients who have severe rheumatoid arthritis.

Takayasu’s Arteritis

Takayasu arteritis affects the aorta (the largest blood vessel in the body) and its branches, which carry blood away from the heart. It typically occurs in Asian women younger than 40.

Wegener’s Granulomatosis

Wegener’s granulomatosis can occur anywhere in the body. It most commonly affects the upper respiratory tract (nose, sinuses, and throat), lungs, and kidneys. It is most common in people who are middle-aged and older. 

Symptoms

Vasculitis can have many different signs and symptoms depending on the type of vasculitis and which organs are involved as well as how severely they are affected. With vasculitis, any number of organs may be involved, so a patient can experience very few signs and symptoms or may be very sick.

There are two types of signs and symptoms that are common among people with vasculitis: those that affect the body in a general way (systemic) and those that affect specific organs or body systems.

Systemic

Systemic signs and symptoms are not specific to a particular part of the body, but affect a person overall, causing general aches, pains, and sickness. Common systemic symptoms include:

• Fever
• Loss of appetite
• Weight loss
• Fatigue (feeling tired) and weakness
• General aches and pains

Organ- or Body System-Specific

These signs and symptoms are specific to a particular organ or body system. The organs and body systems that can be affected include:

• Skin. People may experience a variety of skin changes, including purple or red spots. The changes may look like clusters of small dots, splotches, bruises, or hives. They may be itchy or painless.
• Joints. People can experience aches and arthritis if the joints are affected.
• Lungs. People may experience shortness of breath or even cough up blood. On a chest x ray, lung symptoms may look like pneumonia, even though they are not.
• Gastrointestinal tract. Ulcers in the mouth may be present in some types of vasculitis. Also, abdominal pain or bloody diarrhea can occur in people with vasculitis. In some severe cases, the intestines can rupture.
• Sinuses, nose, and ears. People may experience sinus infections, chronic middle ear infections, ulcers in the nose, or in certain cases there may be hearing loss.
• Eyes. People whose eyes are affected by vasculitis may experience a blurring or loss of vision.
• Brain. People may experience headaches, confusion, changes in behavior, or strokes.
• Nerves. People may experience numbness, tingling, and weakness in various parts of the body. They also may experience symptoms in their limbs, such as loss of feeling or strength in the hands and feet or shooting pains in the arms and legs.

Diagnosis

A variety of tests are used to diagnose vasculitis. The type of test chosen will depend on the signs and symptoms a person has. Some of the tests used in the diagnosis of vasculitis include:

• Blood tests. These may be done to look for abnormal levels of blood cells or antibodies, which could be a sign of inflammation in the body.
• Biopsy. During this test, the doctor takes a small sample of tissue from a blood vessel or one of the affected organs and looks at it under a microscope for inflammation or damage. A biopsy is often the best way to make a firm diagnosis of vasculitis.
• Urine analysis. This test might be done to look for abnormal levels of protein or blood cells in the urine, which could be a sign of vasculitis affecting the kidneys.
• EKG (electrocardiogram). This test measures the rate and regularity of the heartbeat, and is done to see if vasculitis is affecting the heart.
• Echocardiogram. This test uses sound waves to create a moving picture of your heart. Echocardiogram provides information about the size and shape of your heart and how well your heart chambers and valves are functioning. The test also can identify areas of poor blood flow to the heart, areas of heart muscle that are not contracting normally, and previous injury to the heart muscle caused by poor blood flow.
• Chest x ray. A chest x ray takes a picture of the organs and structures inside the chest, including the heart, lungs, and blood vessels. A chest x ray may show abnormal changes if vasculitis is affecting the lungs.
• Pulmonary function testing. These are breathing tests that evaluate how well the lungs are working. These tests are done to see if vasculitis is affecting how the lungs work.
• Abdominal ultrasound. This test uses sound waves to create a picture to look for vasculitis affecting the abdominal organs. It is similar to an echocardiogram.
• Computerized tomography (CT) scan. A CT scan provides a computer generated x-ray image of the internal organs. CT scans can be used to look for vasculitis affecting the abdominal organs or the brain.
• Magnetic resonance imaging (MRI). This test uses powerful magnets and radio waves to make images and can be used to look for a vasculitis affecting the brain.
• Angiography. This test may be done to see the flow of blood through the blood vessels and to determine whether they are blocked. During this test, a dye is injected into the blood vessels, and x-ray pictures of the blood vessels are taken.

Causes

Vasculitis is an inflammation of the blood vessels, but what causes the inflammation is often unknown. It is sometimes a side effect of the body’s immune system response to a recent or chronic infection. It also can be a side effect of the body’s response to a medicine. The body sometimes recognizes a medicine as a foreign substance, and may develop an "allergic reaction" to try to get rid of it.

Vasculitis is sometimes linked to other diseases, such as:

• Autoimmune system diseases. These are diseases that the vasculitis patient may have had for years, in which the body’s immune system mistakenly attacks the body itself. Examples include systemic lupus erythematosus (lupus), rheumatoid arthritis, and scleroderma.
• Blood cancers. These include leukemia and lymphoma.

Treatment

Most cases of vasculitis are treated with prescription medicines.

Goals of Treatment

The main goal of treatment is to stop the inflammation in the affected blood vessels. Most treatments do this by stopping the immune or inflammatory response that caused the vasculitis to occur.

Specific Types of Treatment

There are two types of prescription medicines that are typically used to treat vasculitis: corticosteroid medicines and cytotoxic medicines.

Corticosteroid Medicines

Corticosteroid medicines are often called steroids, though these are not the same kind of steroids that athletes have been reported to use. These medicines are used to reduce the inflammation in the blood vessels. Examples of corticosteroids that the doctor might prescribe include prednisone, prednisolone, and methylprednisolone.

Cytotoxic Medicines

Cytotoxic medicines are typically used to treat cancer, but certain drugs also can be used to treat vasculitis. They may be prescribed in severe cases or in cases in which the patient did not respond to corticosteroids. Sometimes, they are prescribed along with corticosteroids. Cytotoxic medicines work by killing the cells that have caused the inflammation. Examples of these medicines include azathioprine and cyclophosphamide. Doses used for vasculitis are usually lower than those used to treat cancer.

Reference:

National Heart, Lung, and Blood Institute, USA.