Vasculitis

Illustration of a normal artery, narrowed artery, totally occluded artery, and aneurysm. Source: NHLBI

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. The best way to categorize this diverse group of diseases is based on the size of the vessels involved, small vs. medium size vessel, and the organs which are supplied by these vessels.^[1]

When a blood vessel becomes inflamed, it can:

• Narrow, making it more difficult for blood to get through (partial occlusion)
• Close off completely so that blood can not get through at all (complete occlusion)
• More rarely, stretch and weaken so much that a portion bulges outward (an aneurysmal sac or aneurysm) and may possibly burst (ruptured aneurysm)

In the image on the right, 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 of 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.

Other Names

• Arteritis
• Angiitis

Types

Many different disorders 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 they affect and which organs are involved.

Behcet disease

Behcet 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, the genitals, joints, and skin. People with this type of vasculitis are often younger than those with other types; many are in their twenties and thirties. The classic patient is a male in his 20's with painful oral and painful genital ulcers. The HLA-B51 gene is often associated with Behcet disease, but not everyone with the gene develops the disease. Behcet is more common in individuals of Mediterranean, Middle Eastern, or Far Eastern descent, but is usually not found in African Americans.

Buerger disease

Buerger disease typically involves the blood vessels of the hands and feet. It 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 or allergies. They may have had preexisting asthma for three to seven years before the diagnosis of Churg-Strauss syndrome. Other organs are commonly affected such as the kidneys, nervous system, skin, gastrointestinal tract, heart, and eye.

Cryoglobulinemia

This vasculitis is caused by an accumulation of proteins in the bloodstream; these cryoglobulin proteins become insoluble when they are cooled. Common signs and symptoms of cryoglobulinemia are a rash on the lower limbs, arthritis, kidney damage, 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

Also known as temporal arteritis, giant cell arteritis mainly affects the artery in the temple, the temporal artery. This can be a cause of sudden vision loss, and requires immediate medical attention to save the patient's vision. Other common symptoms of giant cell arteritis include headache, fever, and pain in the jaw, shoulders, or hips. It is the most common form of vasculitis that occurs in adults older than 50 years of age. 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 frequent.

Microscopic polyangiitis

Microscopic polyangiitis affects the small blood vessels. It frequently affects the kidneys, skin, and nerves, but it can affect any organ in the body.

Polyarteritis nodosa

Polyarteritis nodosa, which is associated with cryoglobulinemia, frequently affects medium-sized vessels of 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 frequently 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 affects both small and medium-sized blood vessels. It can affect many different organs, including the eyes, skin, hands, and feet. It commonly occurs in patients who have severe rheumatoid arthritis.

Takayasu arteritis

Takayasu arteritis is also known as "pulseless disease" because the pulse or blood pressure may not be obtainable on one side. It affects the aorta (the largest blood vessel in the body) and its branches, which carry blood away from the heart. Heart failure (the inability of the heart to pump blood at sufficient volume for the body's needs) in these patients is not common. The disorder typically occurs in Asian women between the ages of 15 and 30.

Wegener granulomatosis

Wegener Granulomatosis can occur anywhere in the body. It most commonly affects the upper respiratory tract (nose, sinuses, and throat), lungs, and kidneys. Due to the vast number of combinations of symptoms that can occur, it can be difficult to diagnose. People who are middle-aged and older are most susceptible.

Symptoms

Vasculitis has many different symptoms that depend 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 people with the disease can experience very few symptoms, or they may be very sick.

Inflammation of the blood vessels in vasculitis can lead to a loss of blood flow; this can result in damage to the body's organs. Typical symptoms of inflammation (fever, swelling, and weight loss) are common in people who have the disease.

There are two types of symptoms that can occur: symptoms that affect the body in a general way (systemic), and symptoms that affect specific organs or body systems (local).

Systemic symptoms

Systemic 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 the following:

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

Local symptoms

These signs and symptoms affect specific organ or body systems:

• 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.
• Gastrointestinal tract. Ulcers in the mouth, abdominal pain, or bloody diarrhea may be present in some types of 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 sometimes even hearing loss.
• Eyes. People whose eyes are affected by vasculitis may experience a blurring or loss of vision.
• Brain. These effects include headaches, confusion, changes in behavior, or strokes.
• Nerves. Nerve damage causes numbness, tingling, and weakness in various parts of the body. It can also affect the sensation in the limbs, causing loss of feeling or strength in the hands and feet, or shooting pains in the arms and legs.

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. Whatever the cause of the immune response, the end result can be damage to the blood vessels caused by the immune system.^[2]

Vasculitis is sometimes linked to other diseases:

• Autoimmune system diseases. These are diseases that a person with vasculitis 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.

Diagnosis

The diagnosis of vasculitis is based on a medical history, physical exam, signs and symptoms, and laboratory tests.

Specialists involved

Various specialists, depending on the type of vasculitis, can be involved in diagnosis and treatment:

• dermatologist (skin specialist)
• hematologist (blood specialist)
• pulmonologist (lung specialist)
• cardiologist (heart specialist)
• neurologist (nervous system specialist)
• ophthalmologist (eye specialist)
• urologist (urinary tract and urogenital system specialist)
• nephrologist (kidney specialist)
• infectious disease specialist

Tests and procedures

A variety of tests are used to diagnose vasculitis. The type of test chosen depends on the symptoms. Below are some of the tests used in the diagnosis of vasculitis:

• Blood tests. These may be done to look for abnormal levels of blood cells or antibodies, which could be a sign of inflammation.
• 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 the heart. Echocardiogram provides information about the size and shape of the heart and how well the 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 or the aorta (the major artery leaving the heart).
• 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.
• 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.
• MRI. This test uses powerful magnets and radio waves to make images of the inside of the body and can be used to look for vasculitis affecting the brain.
• Angiography. This test may be done to see the flow of blood through the blood vessels and to determine whether it is blocked. During this test, a dye is injected into the blood vessels, and x-ray pictures of the blood vessels are taken.

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.

Corticosteroids

Corticosteroids are a cornerstone of treating most types of vasculitis, and are often used in combination with other immunosuppressive medications. They have a rapid onset of action, and profoundly affect many parts of the immune system as well as most other body systems. 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.

Methotrexate

Methotrexate (MTX) is used to treat forms of vasculitis which, although serious, do not require therapy with cyclophosphamide. MTX often helps patients avoid cyclophosphamide and decreases patients’ needs for steroids. Specific forms of vasculitis treated with MTX include Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, Behcet’s disease, and others.

Cytotoxic medicines

Cyclophosphamide, also called Cytoxan, is classified as a “cytotoxic agent”, because it has a toxic effect on many types of cells (“good” cells as well as “bad”). Cyclophosphamide is one of a number of medications first developed as a chemotherapy drug (a medication used in the treatment of cancer). It was discovered that — in addition to its usefulness in cancer — cyclophosphamide also has a significant ability to suppress the immune system. Thus, the medicine is very effective in the treatment of immunologically–mediated diseases, including some forms of vasculitis. In general, much lower doses of cyclophosphamide are used to treat vasculitis than are used to treat cancer. However, the doses of cyclophosphamide used to treat vasculitis are still high enough to cause a significant number of side–effects, and the drug must be used with great caution.Examples of these medicines include azathioprine (Imuran) and cyclophosphamide (Endoxana, Cytoxan). Doses used for vasculitis are usually lower than those used to treat cancer.^[3]

Prevention

There is currently no known way to prevent vasculitis, but with treatment, the complications of vasculitis can be prevented or delayed.

Living with Vasculitis

The course of vasculitis is unpredictable and depends on the type and severity of the disease. Vasculitis may:

• Go into remission. If caught early it may respond well to treatment and go into remission (symptoms are absent).
• Come back again after the disease has been in remission. These reoccurrences are called flares, and they are generally difficult to predict. Flares can sometimes happen when the doctor takes the patient off of a medicine or alters the dose or type. Also, certain types of vasculitis are more likely to flare than others, and some people are more likely to experience flares than others.
• Remain chronic without remission. In these cases, vasculitis can usually be controlled with continuing medicine treatments for an extended period of time.
• Not respond well to treatment. This can lead to disability or even death. This is rare.

The medicines used to treat vasculitis can have significant side effects. In some cases additional medicines may be needed to reduce these effects. In cases where the symptoms of vasculitis disappear, the doctor may carefully withdraw medicines but will continue to monitor how the disease is progressing. Patients themselves can monitor progress as well as the development of side effects.

Chances of Developing Vasculitis

Risk Factors

Vasculitis can affect anyone, including men, women, and people of all ages. Some types of vasculitis are more likely to affect certain populations than others. Smokers, children, young women, middle-aged adults, and people with chronic hepatitis B and/or C infections are more likely to have certain types of vasculitis. Overall, vasculitis is a fairly rare condition in the general population.

Clinical Trials

• From ClinicalTrials.gov: Current Research
• The Johns Hopkins Vasculitis Center: Vasculitis Center
• The Cleveland Clinic Center for Vasculitis: Center for Vasculitis

Expected Outcome

The outlook for people who have vasculitis varies depending on both the type and severity of the vasculitis. The course of a case of vasculitis can be hard to predict. Treatment is often very effective if vasculitis is diagnosed and treated early. Vasculitis may disappear or go into a period of remission. In some cases, it may be a chronic problem, requiring ongoing treatment, or it may come back (flare) after a period of remission. In more severe cases, treatment may not help, and vasculitis can lead to disability or even death.

References

1. ↑ Jennette JC and Falk RJ. Small-vessel vasculitis. New Eng J Med. 1997 Nov 20;337(21):1512-1523. Abstract
2. ↑ Guillevin L and Dörner T. Vasculitis: mechanisms involved and clinical manifestations. Arthritis Res Ther. 2007;9 (Suppl 2):S9. Abstract | Full Text | PDF
3. ↑ Johns Hopkins Vasculitis Center Vasculitis Treatments

External Links

Central Nervous System Vasculitis Foundation

American Behcet’s Disease Association

Churg-Strauss Syndrome International Support Group

Takayasu’s Arteritis Research Association

Vasculitis Foundation

Lupus Foundation of America Support Groups