Vasculitis

Vasculitis can happen at any age. However, some types of vasculitis are more common among people of certain ages.

• Buerger’s disease usually affects men younger than 45 who smoke or have smoked.
• IgA vasculitis is diagnosed more often in children than adults.
• Giant cell arteritis affects adults 50 years and older and is most common in people who are in their 70s and 80s.
• Kawasaki disease affects only children. It is most common in children younger than 5 years.

Your genes are thought to play a role in some types of vasculitis.

• Behçet’s disease is associated with genes involved in the immune system. It sometimes runs in families.
• Granulomatosis with polyangiitis sometimes runs in families.
• Kawasaki disease may run in families.

Your risk for vasculitis is higher if you engage in certain habits, including:

• Smoking
• Using illegal drugs, such as cocaine

The risk of vasculitis is higher if you take certain medicines, including:

• Hydralazine, used to treat high blood pressure
• Levamisole, used for infections, but also added to most cocaine
• Propylthiouracil, used to treat some thyroid disorders
• Tumor necrosis factor inhibitors, a treatment for some immune diseases

Vasculitis occurs more often in people with certain conditions, including:

• Autoimmune disorders, such as lupus, rheumatoid arthritis, and scleroderma
• Hepatitis B or C, infections that sometimes trigger vasculitis inflammation
• Lymphoma, a cancer of the blood

Some types of vasculitis are more common among people of certain ethnic backgrounds.

• Behçet’s disease is most common in Turkey and is relatively common in other countries in the Mediterranean, the Middle East, Central Asia, China, and Japan. It is relatively uncommon in Northern and Western Europe and the United States.
• Giant cell arteritis is most common in Scandinavia and Minnesota.
• Kawasaki disease is more common among children of Japanese descent.

Some types of vasculitis are more common in men or women.

• Behçet’s disease is more common in men in some countries and more common in women in other countries.
• Buerger’s disease is more common in men.
• Giant cell arteritis affects women two to four times more often than men.
• Microscopic polyangiitis affects men slightly more often than women.

Sometimes the signs and symptoms develop slowly, over months. The signs and symptoms may also develop very quickly, over days or weeks. Some people have general signs and symptoms, such as:

• Fatigue
• Fever
• General aches and pains
• Loss of appetite
• Weight loss

Vasculitis may lead to the following problems in particular organs or parts of your body:

• Ear and nose problems, including sinus infections, inner ear infections, ulcers in the nose, a runny nose, dizziness, ringing in the ears, and hearing loss
• Eye problems, including redness, itching, burning, and changes in vision. Blindness in one eye may be the first sign of giant cell arteritis.
• Gastrointestinal tract problems, such as ulcers in the mouth, esophagus, and stomach; diarrhea; vomiting blood; and abdominal pain
• Genital ulcers
• Headache, scalp tenderness, and pain after chewing
• Heart palpitations
• Joint pain
• Lung problems, including shortness of breath and coughing up blood
• Nerve problems, including numbness, tingling, pain, and weakness in various parts of body; loss of strength in the hands and feet; and shooting pains in the arms and legs
• Skin rashes, purple or red spots or bumps, clusters of small dots, splotches, bruises, hives, and itching
• Swelling of the palms of the hands and soles of the feet; hardening of the hands and feet, which may be painful; pain in the feet or legs; ulcers; and gangrene
• Swollen, dry lips or tongue, or swelling in the mouth and throat

Inflammation from vasculitis can block normal blood flow, which can damage parts of the body. Complications depend on which parts of the body are damaged, and they can be life-threatening. They include:

• Aneurysm or aortic dissection
• Arrhythmia
• Bleeding within the lung
• Blindness. This complication of giant cell arteritis is usually painless and sudden and usually affects only one eye at a time. The risk of blindness with giant cell arteritis is higher for people who have had a stroke or have peripheral artery disease.
• Deafness
• Deep vein thrombosis, a type of venous thromboembolism
• Gangrene
• Heart attack
• High blood pressure
• Low blood pressure
• Ischemic heart disease
• Kidney disease
• Myocarditis, a type of heart inflammation
• Pregnancy complications. Risks to the mother and baby include preeclampsia, the baby growing too slowly inside the womb, loss of the pregnancy, and death of the mother. Complications are usually worse if the disease is active during pregnancy.
• Stroke and transient ischemic attack (TIA). A TIA, also known as a mini-stroke, occurs if blood flow to a portion of the brain is blocked only for a short time.

Depending on the organs affected, your doctor may refer you to one or more of the following specialists:

• Cardiologist, a doctor who specializes in the heart
• Dermatologist, a doctor who specializes in skin
• Infectious disease specialist
• Nephrologist, a doctor who specializes in the kidneys
• Neurologist, a doctor who specializes in the brain and nervous system
• Ophthalmologist, a doctor who specializes in eyes
• Pulmonologist, a doctor who specializes in the lungs
• Rheumatologist, a doctor who specializes in joints, muscles, and autoimmune diseases
• Urologist, a doctor who specializes in the urinary tract and urogenital system

Learn more about the diagnostic tests and procedures your doctor may use to diagnose your vasculitis.

Diagnosis of vasculitis can be difficult. Some types of vasculitis cannot be diagnosed with a test. Instead, your doctor will diagnose you based on your symptoms or complications.

For other types of vasculitis, your doctor may order one or more of the following tests and procedures:

• Biopsy to collect a small sample of your tissue from a specific blood vessel or an organ. A pathologist will study the sample for specific signs of inflammation and tissue damage.
• Blood tests to detect levels of certain blood cells and antibodies in your blood.
• Chest X-ray to find out whether vasculitis is affecting your lungs or your large arteries, such as the aorta or the arteries in the lungs.
• Computed tomography (CT) scan to look for signs of granulomatosis with polyangiitis.
• Echocardiography, an ultrasound test to learn how well the heart is working.
• Pathergy test to help diagnose Behçet’s disease. In this test, a needle pricks the skin, and sometimes a small amount of saline solution may be injected. The test is positive if a red bump or ulcer develops after two days.
• Coronary angiography to look at your blood vessels for damage, inflammation, blockages, or aneurysms.
• Positron electron tomography (PET) scan, a type of nuclear scan, to detect inflammation in the blood vessels.
• Ultrasound to look for signs of inflammation in your blood vessels or organs.
• Urinalysis to check for kidney damage.

To help diagnose vasculitis, your doctor may need to do tests to rule out other medical conditions that have symptoms and complications that resemble vasculitis, including:

• Cancer
• Infectious diseases
• Migraine

Some of the medicines your doctor may prescribe for vasculitis include:

• Corticosteroids to reduce inflammation in your blood vessels. For some types of vasculitis, you will need steroids for months or years. Corticosteroids can affect your bone density and raise your blood sugar and blood pressure levels. Learn about other side effects and risks of corticosteroids.
• Dual endothelin receptor antagonists to block the action of a chemical called endothelin that can reduce blood flow.
• Immunomodulators, such as colchicine, to reduce the inflammation that causes symptoms. Possible side effects can include gastrointestinal problems.
• Immunosuppressive medicines, such as cyclosporine and Mycophenolate mofetil, to suppress or weaken the immune system. Possible side effects of immunosuppressive medicines include an increased risk of infection and birth defects.
• Interferon therapy to help block and reduce inflammation. Interferons are molecules that the immune system normally makes, but they have also been developed as medicines.
• Interleukin antagonists to reduce inflammation by blocking a protein in the body that triggers inflammation.
• Intravenous immunoglobulin (IVIG) to help control the body’s immune and inflammatory response. This medicine also fights infection by introducing purified antibodies from healthy donors into a recipient’s bloodstream. Some people may have a strong negative immune response to IVIG.
• Monoclonal antibodies to help suppress the immune system. Possible side effects include fever-like symptoms, stomach pain, and allergic reactions.
• Non-steroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation in the body. Over-the-counter NSAIDs such as aspirin, ibuprofen, or naproxen may relieve pain for people who have mild vasculitis. One possible side effect is increased bleeding.
• Phosphodiesterase inhibitors to increase blood flow by blocking the action of a particular enzyme in the body. Possible side effects include headaches, heart palpitations, upset stomach, nausea, and vomiting.
• Tumor necrosis factor inhibitors to help suppress the immune system by blocking a protein called tumor necrosis factor alpha.

If medicines do not work to treat vasculitis, your doctor may recommend plasmapheresis or surgery.

• Plasmapheresis to lower plasma antibody levels may sometimes be performed for vasculitis.
• Surgical bypass of the blood vessels may help restore blood flow to some areas in Buerger’s disease.

While you are being treated for vasculitis, you will need to see your doctor regularly.

• Talk to your doctor about any new symptoms and other changes in your health, including side effects of your medicines.
• Your doctor will monitor you for problems associated with medicines used to treat vasculitis, such as corticosteroids.
• If you had Kawasaki disease as a child, you will need follow-up heart testing.

Return to Treatment to review possible treatment options for vasculitis.

To monitor your condition, your doctor may recommend the following tests and procedures.

• Blood tests to look for abnormal levels of certain blood cells and antibodies.
• Cardiac magnetic resonance imaging (MRI) to look for heart and vascular problems caused by vasculitis.
• Chest X-ray to look for any problems in the lungs, heart, and large blood vessels, such as an aortic aneurysm.
• Echocardiography to look for heart problems caused by vasculitis.
• Electrocardiography (EKG) to look for heart rhythm problems caused by vasculitis.
• Myocardial perfusion imaging to look at the blood supply to your heart. It can also be used to look for heart problems caused by vasculitis.
• PET scan to look for aneurysms or heart problems caused by vasculitis.

Most women who have vasculitis have no problems during pregnancy. However, vasculitis can raise the risk to mother and baby.

• If you had Kawasaki disease or another type of vasculitis as a child, tell your doctor if you are planning to become pregnant, as your doctor will want to monitor you for heart problems during pregnancy.
• Some medicines given to people who have vasculitis are dangerous to the developing baby; be sure your doctor knows what you are taking. Medicines may need to be adjusted during pregnancy. Do not stop taking medicine without first talking with your doctor.
• Vasculitis raises your risk for high blood pressure during pregnancy. Your doctor will want to monitor your blood pressure closely.

After vasculitis is treated and goes into remission, you may have flares, which are a repeat occurrence of signs and symptoms. You may have different symptoms than when you first had vasculitis.

Part of the goal of vasculitis treatment is avoiding flares.

• Flares may be treated with some of the same medicines used for your initial treatment, including corticosteroids.
• If your vasculitis goes into remission, your doctor may carefully stop your medicines. However, your doctor will still need to monitor you for flares.

To help you prevent or treat some of the complications of vasculitis, your doctor may recommend medicines, healthy lifestyle changes, or procedures.

Medicines may include:

• Anticlotting medicines to treat blood clots or prevent blood clots from forming. You may need them if you have an aneurysm.
• Beta blockers to lower blood pressure. You may need them if you have an aneurysm.
• Statins to control or lower high blood cholesterol levels.

Healthy lifestyle changes include:

• Adopting a heart-healthy lifestyle.
• Avoiding illegal drugs, including cocaine.
• Quitting smoking and tobacco. Visit Smoking and Your Heart and the National Heart, Lung, and Blood Institute’s Your Guide to a Healthy Heart. Although these resources focus on heart health, they include basic information about how to quit smoking. For free help and support to quit smoking, you may call the National Cancer Institute’s Smoking Quitline at 1-877-44U-QUIT (1-877-448-7848).

If you have an aneurysm, it can lead to a serious complication of a dissection or rupture. Vasculitis also can lead to serious cardiovascular complications, such as heart attack or stroke.

If you think that you are or someone else is having symptoms of one of these conditions, call 9-1-1 immediately. Every minute matters.

Aneurysm rupture or dissection

Signs and symptoms of a dissection or rupture may include:

• Light-headedness
• Paleness
• Rapid heart rate
• Sudden, severe pain in your abdomen, chest, or back, which can travel upward or downward

Heart attack

Signs of heart attack include mild or severe chest pain or discomfort in the center of the chest or upper abdomen that lasts for more than a few minutes or goes away and comes back. It can feel like pressure, squeezing, fullness, heartburn, or indigestion. There may also be pain down the left arm. Women may also have chest pain and pain down the left arm, but they are more likely to have symptoms such as shortness of breath, nausea, vomiting, unusual tiredness, and pain in the back, shoulders, or jaw.

Read more about the signs and symptoms of a heart attack.

Stroke

If you think someone may be having a stroke or transient ischemic attack (TIA), act F.A.S.T. and do the following simple test.

F—Face: Ask the person to smile. Does one side of the face droop?
A—Arms: Ask the person to raise both arms. Does one arm drift downward?
S—Speech: Ask the person to repeat a simple phrase. Is his or her speech slurred or strange?
T—Time: If you observe any of these signs, call 9-1-1 immediately. Early treatment is essential.

Read more about the signs and symptoms of a stroke.