What Is Vasculitis?
Vasculitis is an inflammation of the blood vessels
in the body. In vasculitis, the bodys immune system mistakenly attacks
the bodys 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 bodys blood
vessels. These include arteries, veins, and capillaries. Arteries are vessels
that carry blood from the heart to the bodys 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 cant 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.
The disruption in blood flow from inflammation can
damage the bodys organs. Specific signs and symptoms depend on which
organ has been damaged and the extent of the damage. Typical signs and symptoms
of inflammation (fever, swelling, and a general sense of feeling ill) are
common among people with vasculitis.
Outlook
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.
Much is still unknown about vasculitis. There are
many different types of vasculitis, but overall it is a fairly rare condition
in the general population. Additional research is needed to learn more about
the various types of vasculitis and their causes, treatments, and remission
patterns.
Types of Vasculitis
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.
Behcets Disease
Behcets 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 Behcets
disease, but not everyone with the gene will develop the disease. Behcets
is more common in individuals of Mediterranean, Middle Eastern, or Far Eastern
descent, but is usually not found in Blacks.
Buergers Disease
Buergers disease typically affects blood flow
to the hands and feet. Buergers 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
(KRI-o-GLOB-u-luh-NE-me-uh) 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 (POL-e-an-je-i-tis) most
commonly affects the kidneys, skin, and nerves, but it can affect any organ in
the body.
Polyarteritis Nodosa
Polyarteritis nodosa (POL-e-ar-ter-i-tis no-DO-suh)
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 (POL-e-mi-AL-je-a ROO-ma-ti-kuh) 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.
Takayasus Arteritis
Takayasu (Tock-e-AH-sue) arteritis (ar-ter-I-tis)
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.
Wegeners Granulomatosis
Wegeners granulomatosis (GRAN-u-lo-ma-TO-sis)
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.
Other Names for Vasculitis
What Causes Vasculitis?
Vasculitis is an inflammation of the blood vessels,
but what causes the inflammation is often unknown. It is sometimes a side
effect of the bodys immune system response to a recent or chronic
infection. It also can be a side effect of the bodys 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 bodys
immune system mistakenly attacks the body itself. Examples include systemic
lupus erythematosus (lupus), rheumatoid arthritis, and scleroderma.
- Blood cancers. These include leukemia and
lymphoma.
Who Is At Risk for Vasculitis?
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. Examples of populations that might be more
affected by certain types of vasculitis include smokers, children, young women,
middle-aged adults, and people with chronic hepatitis B and/or C infections.
What Are the Signs and Symptoms of Vasculitis?
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.
How Is Vasculitis Diagnosed?
The diagnosis of vasculitis is based on a
persons medical history, physical exam, signs and symptoms, and
laboratory tests.
Specialists Involved
A person with vasculitis may be referred to various
specialists, including:
- A dermatologist (skin specialist)
- A hematologist (blood specialist)
- A pulmonologist (lung specialist)
- A cardiologist (heart specialist)
- A neurologist (nervous system specialist)
- An ophthalmologist (eye specialist)
- A urologist (urinary tract and urogenital system
specialist)
- A nephrologist (kidney specialist)
- An infectious disease specialist
Diagnostic Tests and Procedures
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.
How Is Vasculitis Treated?
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.
How Can Vasculitis Be Prevented?
There is currently no known way to prevent
vasculitis, but with treatment, the complications of vasculitis can be
prevented or delayed.
Living With Vasculitis
General Information About Outcomes
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.
- Reoccur. 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 patients 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.
Ongoing Health Care Needs
The medicines used to treat vasculitis can have
significant side effects. Your doctor may adjust the type or dose of medicine
you take to lessen the side effects.
In cases in which remission occurs, the doctor may
carefully withdraw medicines but still require careful monitoring of flares.
Patients who continue taking medicines should stay under the careful watch of
their doctor to monitor and control side effects. Patients also should always
monitor their health, side effects, and symptoms and discuss any changes with
their doctor.
Support Groups
Key Points
- Vasculitis is an inflammation of blood vessels in
the body. In vasculitis, the bodys immune system mistakenly attacks the
bodys own blood vessels, causing them to become inflamed.
- The inflammation can cause disruption of blood
flow to parts of the body, which can lead to tissue damage or even death.
- Vasculitis can affect any blood vessel or organ
in the body.
- The exact cause of the inflammation in vasculitis
is unknown, but it is sometimes a side effect of the bodys normal immune
system response.
- Vasculitis can affect people of all ages and
either gender. Some types of vasculitis are more likely to occur in certain
populations than others.
- There are many types of vasculitis, and the signs
and symptoms vary widely in type and severity. They may be specific and affect
a particular organ, or they may be nonspecific, causing general aches, pains,
and fatigue (feeling tired).
- Laboratory tests are usually performed to confirm
a diagnosis of vasculitis. There are many different tests that may be run,
depending on the signs and symptoms present and where they are in the
body.
- Vasculitis is typically treated with prescription
medicines with the goal of stopping the inflammation in the blood vessels and
relieving symptoms.
- Corticosteroid medicines or cytotoxic medicines
are typically the drugs used to treat vasculitis.
- The course of a case of vasculitis is often
difficult to predict. Vasculitis may go into remission, reoccur, remain
chronic, or, in rare cases, lead to severe disability or death.
Links to Other Information About Vasculitis
Non-NHLBI Resources
Clinical Trials
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