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Vasculitis is the general term for inflammation of blood vessels. There are many forms of vasculitis. Each is characterized by:
- The type of vessels that are affected— large, medium or small arteries
- The distribution of the inflammation throughout the body.
Any blood vessel, from the largest, the aorta. to the smallest, skin capillaries,may be affected. Vasculitis may also be associated with other systemic diseases including:
- Lupus erythematosis (lupus)
- Other autoimmune diseases
- Collagen vascular or connective tissue disorders.
Some of the most common types of vasculitis are:
- Raynaud’s Syndrome (Raynaud's disease and Raynaud's phenomenon)
- Buerger’s Disease (Thromboangitis Obliterans)
- Takayasu’s Arteritis
“Inflammation” refers to the body’s response to injury, including injury to the blood vessels. Inflammation may involve pain, redness, warmth, swelling, and loss of function in the affected tissues.
In vasculitis, inflammation can lead to serious problems. Complications depend on which blood vessels, organs, or other body systems are affected.
Vasculitis can affect any of the body's blood vessels. These include arteries, veins, and capillaries. Arteries carry blood from your heart to your body's organs. Veins carry blood from your organs and limbs back to your heart. Capillaries connect the small arteries and veins.
If a blood vessel is inflamed, it can narrow or close off. This limits or prevents blood flow through the vessel. Rarely, the blood vessel will stretch and weaken, causing it to bulge. This bulge is known as an aneurysm (AN-u-rism).
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 inflamed, narrowed artery with decreased blood flow. The inset image shows a cross-section of the inflamed artery. Figure C shows an inflamed, blocked (occluded) artery and scarring on the artery wall. The inset image shows a cross-section of the blocked artery. Figure D shows an artery with an aneurysm. The inset image shows a cross-section of the artery with an aneurysm.
The disruption in blood flow caused by inflammation can damage the body's organs. Signs and symptoms depend on which organs have been damaged and the extent of the damage.
Typical symptoms of inflammation, such as fever and general aches and pains, are common among people who have vasculitis.
There are many types of vasculitis, but overall the condition is rare. If you have vasculitis, the outlook depends on:
- The type of vasculitis you have
- Which organs are affected
- How quickly the condition worsens
- The severity of the condition
Treatment often works well if it’s started early. In some cases, vasculitis may go into remission. "Remission" means the condition isn't active, but it can come back, or "flare," at any time.
Sometimes vasculitis is chronic (ongoing) and never goes into remission. Long-term treatment with medicines often can control the signs and symptoms of chronic vasculitis.
Rarely, vasculitis doesn't respond well to treatment. This can lead to disability and even death.
Much is still unknown about vasculitis. However, researchers continue to learn more about the condition and its various types, causes, and treatments.
Types of Vasculitis
There are many types of vasculitis. Each type involves inflamed blood vessels. However, most types differ in whom they affect and the organs that are involved.
The types of vasculitis often are grouped based on the size of the blood vessels they affect.
Mostly Large Vessel Vasculitis
These types of vasculitis usually, but not always, affect the body’s larger blood vessels.
Behçet's (beh-CHET'S) disease can cause recurrent, painful ulcers (sores) in the mouth, ulcers on the genitals, acne-like skin lesions, and eye inflammation called uveitis (u-ve-I-tis).
The disease occurs most often in people aged 20 to 40. Men are more likely to get it, but it also can affect women. Behçet's disease is more common in people of Mediterranean, Middle Eastern, and Far Eastern descent, although it rarely affects Blacks.
Researchers believe that a gene called the HLA-B51 gene may play a role in Behçet's disease. However, not everyone who has the gene gets the disease.
Cogan's syndrome can occur in people who have a systemic (sis-TEH-mik) vasculitis that affects the large blood vessels, especially the aorta and aortic valve. The aorta is the main artery that carries oxygen-rich blood from the heart to the body.
A systemic vasculitis is a type of vasculitis that affects you in a general or overall way.
Cogan's syndrome can lead to eye inflammation called interstitial keratitis (in-ter-STISH-al ker-ah-TI-tis). The syndrome also can cause hearing changes, including sudden deafness.
Giant Cell Arteritis
Giant cell arteritis (ar-ter-I-tis) usually affects the temporal artery, an artery on the side of your head. This condition also is called temporal arteritis. Symptoms of this condition can include headaches, scalp tenderness, jaw pain, blurred vision, double vision, and acute (sudden) vision loss.
Giant cell arteritis is the most common form of vasculitis in adults older than 50. It's more likely to occur in people of Scandinavian origin, but it can affect people of any race.
Polymyalgia rheumatica (POL-e-my-AL-jah RU-ma-ti-kuh), or PMR, commonly affects the large joints in the body, such as the shoulders and hips. PMR typically causes stiffness and pain in the muscles of the neck, shoulders, lower back, hips, and thighs.
PMR usually occurs by itself, but 10–20 percent of people who have PMR also develop giant cell arteritis. Also, about half of the people who have giant cell arteritis may develop PMR.
Takayasu's (tock-e-AH-sues) arteritis affects medium- and large-sized arteries, particularly the aorta and its branches. The condition sometimes is called aortic arch syndrome.
Though rare, Takayasu's arteritis mainly affects teenage girls and young women. The condition is most common in Asians, but it can affect people of all races.
Takayasu's arteritis is a systemic disease. A systemic disease is one that affects you in a general or overall way.
Symptoms of Takayasu's arteritis may include tiredness and a sense of feeling unwell, fever, night sweats, sore joints, loss of appetite, and weight loss. These symptoms usually occur before other signs develop that point to arteritis.
Mostly Medium Vessel Vasculitis
These types of vasculitis usually, but not always, affect the body’s medium-sized blood vessels.
Buerger's disease, also known as thromboangiitis (THROM-bo-an-jee-I-tis) obliterans, typically affects blood flow to the hands and feet. In this disease, the blood vessels in the hands and feet tighten or become blocked. As a result, less blood flows to the affected tissues, which can lead to pain and tissue damage.
Rarely, Buerger's disease also can affect blood vessels in the brain, abdomen, and heart. The disease usually affects men aged 20 to 40 of Asian or Eastern European descent. The disease is strongly linked to cigarette smoking.
Symptoms of Buerger's disease include pain in the calves or feet when walking or pain in the forearms and hands with activity. Other symptoms include blood clots in the surface veins of the limbs and Raynaud's phenomenon.
In severe cases, ulcers may develop on the fingers and toes, leading to gangrene. The term "gangrene" refers to the death or decay of body tissues.
Surgical bypass of the blood vessels may help restore blood flow to some areas. Medicines generally don’t work well to treat Buerger’s disease. The best treatment is to stop using tobacco of any kind.
Central Nervous System Vasculitis
Central nervous system (CNS) vasculitis usually occurs as a result of a systemic vasculitis. A systemic vasculitis is one that affects you in a general or overall way.
Very rarely, vasculitis affects only the brain and/or spinal cord. When it does, the condition is called isolated vasculitis of the central nervous system or primary angiitis (an-jee-I-tis) of the central nervous system.
Symptoms of CNS vasculitis include headaches, problems thinking clearly, changes in mental function, or stroke-like symptoms, such as muscle weakness and paralysis (an inability to move).
Kawasaki (KAH-wah-SAH-ke) disease is a rare childhood disease in which the walls of the blood vessels throughout the body become inflamed. The disease can affect any blood vessel in the body, including arteries, veins, and capillaries.
Kawasaki disease also is known as mucocutaneous (myu-ko-kyu-TA-ne-as) lymph node syndrome. This is because the disease is associated with redness of the mucous membranes in the eyes and mouth, redness of the skin, and enlarged lymph nodes. (Mucous membranes are tissues that line some organs and body cavities.)
Sometimes the disease affects the coronary arteries, which carry oxygen-rich blood to the heart. As a result, a small number of children who have Kawasaki disease may have serious heart problems.
Polyarteritis nodosa (POL-e-ar-ter-I-tis no-DO-suh) can affect many parts of the body. This disorder often affects the kidneys, the digestive tract, the nerves, and the skin.
Symptoms often include fever, a general feeling of being unwell, weight loss, and muscle and joint aches, including pain in the calf muscles that develops over weeks or months.
Other signs and symptoms include anemia (a low red blood cell count), a lace- or web-like rash, bumps under the skin, and stomach pain after eating.
Researchers believe that this type of vasculitis is very rare, although the symptoms can be similar to those of other types of vasculitis. Some cases of polyarteritis nodosa seem to be linked to hepatitis B or C infections.
Mostly Small Vessel Vasculitis
These types of vasculitis usually, but not always, affect the body’s small blood vessels.
Eosinophilic Granulomatosis With Polyangiitis
Eosinophilic granulomatosis (E-o-Sin-o-Fil-ik GRAN-u-lo-ma-TO-sis) with polyangiitis (POL-e-an-jee-I-tis), or EGPA is a very rare disorder that causes blood vessel inflammation. The disorder is also known as Churg-Strauss syndrome or allergic angiitis and granulomatosis.
EGPA can affect many organs, including the lungs, skin, kidneys, nervous system, and heart. Symptoms can vary widely. They may include asthma, higher than normal levels of white blood cells in the blood and tissues, and abnormal lumps known as granulomas (gran-yu-LO-mas).
Cryoglobulinemic (KRI-o-GLOB-u-luh-NE-mik)vasculitis occurs when abnormal immune proteins (cryoglobulins) thicken the blood and impair blood flow. This causes pain and damage to the skin, joints, peripheral nerves, kidneys, and liver.
Cryoglobulins are abnormal immune proteins in the blood that clump together and thicken the blood plasma. Cryoglobulins can be detected in the laboratory by exposing a sample of blood to cold temperature (below normal body temperature). In cold temperatures, the immune proteins form clumps; but when the blood is rewarmed, the clumps dissolve.
The cause of cryoglobulinemic vasculitis is not always known. In some cases, it is associated with other conditions such as lymphoma, multiple myeloma, connective tissue diseases, and infection (particularly hepatitis C infection).
In IgA (immunoglobulin A) vasculitis (also known as Henoch-Schonlein purpura), abnormal IgA deposits develop in small blood vessels in the skin, joints, intestines, and kidneys. IgA a type of antibody (a protein) that normally helps defend the body against infections.
Symptoms of IgA vasculitis include a bruise-like, reddish-purple rash, most often seen on the buttocks, legs, and feet (but can be anywhere on the body); abdominal pain; swollen and painful joints; and blood in the urine. People with IgA vasculitis do not necessarily have all of the symptoms, but nearly all will have the characteristic rash.
IgA vasculitis is most often seen in children between 2 and 11 years of age, but it can affect people of all ages. More than 75 percent of the cases of IgA vasculitis follow an upper respiratory tract infection, a throat infection, or a gastrointestinal infection.
Most people with IgA vasculitis are well within 1 to 2 months and do not have any lasting problems. In rare cases symptoms can last longer or come back. All IgA vasculitis patients should have a full evaluation by a medical professional.
Hypersensitivity vasculitis affects the skin. This condition also is known as allergic vasculitis, cutaneous (ku-TA-ne-us) vasculitis, or leukocytoclastic (LU-ko-si-TOE-klas-tic) vasculitis.
A common symptom is red spots on the skin, usually on the lower legs. For people who are bedridden, the rash appears on the lower back.
An allergic reaction to a medicine or infection often causes this type of vasculitis. Stopping the medicine or treating the infection usually clears up the vasculitis. However, some people may need to take anti-inflammatory medicines, such as corticosteroids, for a short time. These medicines help reduce inflammation.
Microscopic polyangiitis (POL-e-an-jee-I-tis) affects small blood vessels, particularly those in the kidneys and lungs. The disease mainly occurs in middle-aged people; it affects men slightly more often than women.
The symptoms often aren't specific, and they can begin gradually with fever, weight loss, and muscle aches. Sometimes the symptoms come on suddenly and progress quickly, leading to kidney failure.
If the lungs are affected, coughing up blood may be the first symptom. Sometimes microscopic polyangiitis occurs with a vasculitis that affects the intestinal tract, the skin, and the nervous system.
The signs and symptoms of microscopic polyangiitis are similar to those of Wegener's granulomatosis (another type of vasculitis). However, microscopic polyangiitis usually doesn't affect the nose and sinuses or cause abnormal tissue formations in the lungs and kidneys.
The results of certain blood tests can suggest inflammation. These results include a higher than normal erythrocyte sedimentation rate (ESR); lower than normal hemoglobin (HEE-muh-glow-bin) and hematocrit (hee-MAT-oh-crit) levels (which suggest anemia); and higher than normal white blood cell and platelet (PLATE-let) counts.
Also, more than half of the people who have microscopic polyangiitis have certain antibodies (proteins) in their blood. These antibodies are called antineutrophil cytoplasmic autoantibodies (ANCA). ANCA also occur in people who have Wegener's granulomatosis.
Testing for ANCA can't be used to diagnose either of these two types of vasculitis. However, testing can help evaluate people who have vasculitis-like symptoms.
What Causes Vasculitis?
Vasculitis occurs if your immune system attacks your blood vessels by mistake. What causes this to happen isn't fully known.
A recent or chronic (ongoing) infection may prompt the attack. Your body also may attack its own blood vessels in reaction to a medicine.
Sometimes an autoimmune disorder triggers vasculitis. Autoimmune disorders occur if the immune system makes antibodies (proteins) that attack and damage the body's own tissues or cells. Examples of these disorders include lupus, rheumatoid arthritis, and scleroderma. You can have these disorders for years before developing vasculitis.
Vasculitis also may be linked to certain blood cancers, such as leukemia and lymphoma.
Who Is at Risk for Vasculitis?
Vasculitis can affect people of all ages and races and both sexes. Some types of vasculitis seem to occur more often in people who:
- Have certain medical conditions, such as chronic hepatitis B or C infection
- Have certain autoimmune diseases, such a lupus, rheumatoid arthritis, and scleroderma
What Are the Signs and Symptoms of Vasculitis?
The signs and symptoms of vasculitis vary. They depend on the type of vasculitis you have, the organs involved, and the severity of the condition. Some people may have few signs and symptoms. Other people may become very sick.
Sometimes the signs and symptoms develop slowly, over months. Other times, the signs and symptoms develop quickly, over days or weeks.
Systemic Signs and Symptoms
Systemic signs and symptoms are those that affect you in a general or overall way. Common systemic signs and symptoms of vasculitis are:
- Loss of appetite
- Weight loss
- Fatigue (tiredness)
- General aches and pains
Organ- or Body System-Specific Signs and Symptoms
Vasculitis can affect specific organs and body systems, causing a range of signs and symptoms.
If vasculitis affects your skin, you may notice skin changes. For example, you may have purple or red spots or bumps, clusters of small dots, splotches, bruises, or hives. Your skin also may itch.
If vasculitis affects your joints, you may ache or develop arthritis in one or more joints.
If vasculitis affects your lungs, you may feel short of breath. You may even cough up blood. The results from a chest x ray may show signs that suggest pneumonia, even though that may not be what you have.
If vasculitis affects your gastrointestinal tract, you may get ulcers (sores) in your mouth or have stomach pain.
In severe cases, blood flow to the intestines can be blocked. This can cause the wall of the intestines to weaken and possibly rupture (burst). A rupture can lead to serious problems or even death.
Sinuses, Nose, Throat, and Ears
If vasculitis affects your sinuses, nose, throat, and ears, you may have sinus or chronic (ongoing) middle ear infections. Other symptoms include ulcers in the nose and, in some cases, hearing loss.
If vasculitis affects your eyes, you may develop red, itchy, burning eyes. Your eyes also may become sensitive to light, and your vision may blur. Rarely, certain types of vasculitis may cause blindness.
If vasculitis affects your brain, symptoms may include headaches, problems thinking clearly, changes in mental function, or stroke-like symptoms, such as muscle weakness and paralysis (an inability to move).
If vasculitis affects your nerves, you may have numbness, tingling, and weakness in various parts of your body. You also may have a loss of feeling or strength in your hands and feet and shooting pains in your arms and legs.
How Is Vasculitis Diagnosed?
Your doctor will diagnose vasculitis based on your signs and symptoms, your medical history, a physical exam, and test results.
Diagnostic Tests and Procedures
Many tests are used to diagnose vasculitis.
Blood tests can show whether you have abnormal levels of certain blood cells and antibodies (proteins) in your blood. These tests may look at:
- Hemoglobin and hematocrit. A low hemoglobin or hematocrit level suggests anemia, a complication of vasculitis. Vasculitis can interfere with the body's ability to make enough red blood cells. Vasculitis also can be linked to increased destruction of red blood cells.
- Antineutrophil cytoplasmic antibodies (ANCA). These antibodies are present in people who have certain types of vasculitis.
- Erythrocyte sedimentation rate (ESR). A high ESR may be a sign of inflammation in the body.
- The amount of C-reactive protein (CRP) in your blood. A high CRP level suggests inflammation.
A biopsy often is the best way for your doctor to make a firm diagnosis of vasculitis. During a biopsy, your doctor will take a small sample of your body tissue to study under a microscope. He or she will take the tissue sample from a blood vessel or an organ.
A pathologist will study the sample for signs of inflammation or tissue damage. A pathologist is a doctor who specializes in identifying diseases by studying cells and tissues under a microscope.
People who have vasculitis should have their blood pressure checked routinely. Vasculitis that damages the kidneys can cause high blood pressure.
For this test, you'll provide a urine sample for analysis. This test detects abnormal levels of protein or blood cells in the urine. Abnormal levels of these substances can be a sign of vasculitis affecting the kidneys.
An EKG is a simple, painless test that records the heart's electrical activity. You might have this test to show whether vasculitis is affecting your heart.
Echocardiography is a painless test that uses sound waves to create a moving picture of your heart. The test gives information about the size and shape of your heart and how well your heart chambers and valves are working.
Chest X Ray
A chest x ray is a painless test that creates pictures of the structures inside your chest, such as your heart, lungs, and blood vessels. Abnormal chest x-ray results may show whether vasculitis is affecting your lungs or your large arteries (such as the aorta or the pulmonary arteries).
Lung Function Tests
Lung function tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood.
Lung function tests can help your doctor find out whether airflow into and out of your lungs is restricted or blocked.
An abdominal ultrasound uses sound waves to create a picture of the organs and structures in your abdomen. The picture may show whether vasculitis is affecting your abdominal organs.
Computed Tomography Scan
A computed tomography (to-MOG-rah-fee) scan, or CT scan, is a type of x ray that creates more detailed pictures of your internal organs than a standard x ray. The results from this test can show whether you have a type of vasculitis that affects your abdominal organs or blood vessels.
Magnetic Resonance Imaging
A magnetic resonance imaging (MRI) test uses radio waves, magnets, and a computer to create detailed pictures of your internal organs.
Other Advanced Imaging Techniques
Several new imaging techniques are now being used to help diagnose vasculitis. Duplex ultrasonography combines an image of the structure of the blood vessel with a color image of the blood flow through that vein or artery. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) identifies areas that show higher glucose metabolism leading to problems in the blood vessels.
Angiography (an-jee-OG-ra-fee) is a test that uses dye and special x rays to show blood flowing through your blood vessels.
The dye is injected into your bloodstream. Special x-ray pictures are taken while the dye flows through your blood vessels. The dye helps highlight the vessels on the x-ray pictures.
How Is Vasculitis Treated?
Treatment for vasculitis will depend on the type of vasculitis you have, which organs are affected, and the severity of the condition.
People who have severe vasculitis are treated with prescription medicines. Rarely, surgery may be done. People who have mild vasculitis may find relief with over-the-counter pain medicines, such as acetaminophen, aspirin, ibuprofen, or naproxen.
The main goal of treating vasculitis is to reduce inflammation in the affected blood vessels. This usually is done by reducing or stopping the immune response that caused the inflammation.
Types of Treatment
Common prescription medicines used to treat vasculitis include corticosteroids and cytotoxic medicines.
Corticosteroids help reduce inflammation in your blood vessels. Examples of corticosteroids are prednisone, prednisolone, and methylprednisolone.
Doctors may prescribe cytotoxic medicines if vasculitis is severe or if corticosteroids don't work well. Cytotoxic medicines kill the cells that are causing the inflammation. Examples of these medicines are azathioprine, methotrexate, and cyclophosphamide.
Your doctor may prescribe both corticosteroids and cytotoxic medicines.
Other treatments may be used for certain types of vasculitis. For example, the standard treatment for Kawasaki disease is high-dose aspirin and immune globulin. Immune globulin is a medicine that’s injected into a vein.
Certain types of vasculitis may require surgery to remove aneurysms that have formed as a result of the condition. (An aneurysm is an abnormal bulge in the wall of a blood vessel.)
How Can Vasculitis Be Prevented?
You can't prevent vasculitis. However, treatment can help prevent or delay the complications of vasculitis.
People who have severe vasculitis are treated with prescription medicines. Rarely, surgery may be done. People who have mild vasculitis may find relief with over-the-counter pain medicines, such as acetaminophen, aspirin, ibuprofen, or naproxen.
Living With Vasculitis
The outcome of vasculitis is hard to predict. It will depend on the type of vasculitis you have, which organs are affected, and the severity of the condition.
If vasculitis is diagnosed early and responds well to treatment, it may go away or go into remission. "Remission" means the condition isn't active, but it can come back, or "flare," at any time.
Flares can be hard to predict. You may have a flare when you stop treatment or change your treatment. Some types of vasculitis seem to flare more often than others. Also, some people have flares more often than others.
Sometimes vasculitis is chronic (ongoing) and never goes into remission. Long-term treatment with medicines often can control chronic vasculitis, but no cure has been found. Rarely, vasculitis doesn't respond well to treatment. This can lead to disability or even death.
The medicines used to treat vasculitis can have side effects. For example, long-term use of corticosteroids may lead to weight gain, diabetes, weakness, a decrease in muscle size, and osteoporosis (a bone-thinning condition). Long-term use of these medicines also may increase your risk of infection.
Your doctor may adjust the type or dose of medicine you take to lessen or prevent the side effects. If your vasculitis goes into remission, your doctor may carefully withdraw your medicines. However, he or she will still need to carefully watch you for flares.
While you're being treated for vasculitis, you'll need to see your doctor regularly. Talk with your doctor about any new symptoms and other changes in your health, including side effects of your medicines.
Emotional Issues and Support
Living with a chronic condition may cause fear, anxiety, depression, and stress. Talk about how you feel with your health care team. Talking to a professional counselor also can help. If you’re very depressed, your doctor may recommend medicines or other treatments that can improve your quality of life.
Joining a patient support group may help you adjust to living with vasculitis. You can see how other people who have the same symptoms have coped with them. Talk with your doctor about local support groups or check with an area medical center.
Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.