What Is Vasculitis?
Vasculitis, also called angiitis or arteritis, is an autoimmune disease that affects your blood vessels, organs, and tissues. Your vessels swell and narrow, which makes it harder for your blood to flow to your tissues and organs. Some vessels could close entirely.
When too little blood reaches your organs and tissues, they can become damaged.
Vasculitis Symptoms
Vasculitis symptoms can show up in many ways, depending on what part of your body is affected. Still, some general symptoms include:
- Fever
- Weight loss
- Loss of appetite
- Fatigue
- Headache
- General aches and pains
Symptoms related to specific areas of your body include:
- Eyes.Your first sign of vasculitis might be red, itchy, or burning eyes. You could also see double and have temporary or permanent blindness in one or both eyes.
- Skin. You might get rashes, lumps, or open sores if vasculitis affects blood vessels going to your skin.
- Nerves. If your nerves don't get enough blood, you could feel numbness, tingling, pain, and weakness.
- Brain. Vasculitis in your brain may cause a stroke.
- Heart. You could have heart palpitations or even a heart attack if it affects your heart.
- Kidneys. Inflammation in the vessels that supply blood to your kidneys can lead to kidney failure.
- Digestive system. You may feel pain after you eat if vasculitis affects your stomach or intestines. You could also see blood in your stool.
- Ears. Vasculitis can cause your ears to ring. It could also cause dizziness or sudden loss of hearing. You might also get inner ear infections.
- Hands and feet. Vasculitis can cause numbness or weakness in your hands or feet, along with swollen or hardened palms and soles.
- Lungs. If vasculitis affects your lungs, you could have shortness of breath or maybe cough up blood.
- Genitals. Vasculitis in this area can cause ulcers or open sores.
- Nose. Along with sinus infections and a runny nose, you could also get blisters in your nose.
- Mouth. Vasculitis can make your lips and tongue swollen and dry, or your mouth and throat swell.
Vasculitis Types
Vasculitis is the general term for several conditions that cause blood vessel inflammation. Doctors organize vasculitis into types based on the size of the blood vessels involved. All types of vasculitis can affect anyone, but some are more common in certain age groups.
- Systemic vasculitis is inflammation of your blood vessel walls, which can happen anywhere in your body.
- Exercise-induced vasculitis is a type of small-vessel vasculitis. It restricts vessels in your lower legs after you do intense exercise like running or hiking, particularly in hot weather. Women over 50 get it most often. Symptoms include rashes on your legs that go away in a few days.
- Urticarial vasculitis affects your skin's small blood vessels. The inflammation usually causes patches and hives that can itch, burn, and discolor your skin. If it gets more serious, it may damage other organs, too.
- Leukocytoclastic vasculitis results when waste from immune cells in the walls of your small blood vessels causes inflammation. When the damaged blood vessels become leaky, they cause raised spots on your skin, usually your legs. Most of the time, it affects only your skin. But it can spread to other organs if it's serious.
- ANCA vasculitis targets a certain type of white blood cell in your body and tells these cells to attack small blood vessels. When the blood vessels are invaded, they become swollen and inflamed. ANCA vasculitis can happen in many parts of your body. The inflammation causes different symptoms, depending on where it is.
- IGA vasculitis is the most common type of vasculitis in children. It causes inflammation and bleeding of small blood vessels in your skin, joints, intestines, and kidneys. The most common symptom is a raised skin rash, usually on your legs or buttocks, that looks like bruises. But if IGA vasculitis affects other organs, you could have stomach or joint pain, swelling, and kidney inflammation.
- Cutaneous vasculitis is when you have inflammation and damage to your skin's blood vessels. It's the most common vasculitis doctors see. It shows up as raised patches on your skin.
- Central nervous system (CNS) vasculitis happens when the blood vessel walls in your brain and spine become inflamed. Many conditions can cause it, though your immune system often plays a role. While it's one of the more serious types of vasculitis, it is treatable.
- Rheumatoid vasculitis is a complication of rheumatoid arthritis (RA) that happens when the inflammation that causes joint pain and damage also damages your blood vessels. Rheumatoid vasculitis causes your small- and medium-sized blood vessels to become inflamed and narrow. It most often shows up in skin, nerves, fingers, and toes.
- Other types of vasculitis include giant cell arteritis, polyarteritis nodosa, Takayasu arteritis, Behçet’s disease, and Kawasaki disease.
Vasculitis Causes
Doctors don’t know exactly what causes many cases of vasculitis. But there are some possible triggers:
- Autoimmune diseases like RA, lupus, or Sjögren's syndrome
- Infections, such as hepatitis B and hepatitis C, that set off an unusual immune system reaction that damages your blood vessels
- Allergic reactions to medications
- Certain blood cancers, like leukemia and lymphoma
Vasculitis Risk Factors
While anyone can get vasculitis, some things can raise your chances of having certain types of the condition, including:
- Your age. Some types are more common in older people, while others, such as Kawasaki disease, most often affect children.
- A family history of a particular type of vasculitis
- Cocaine use
- Smoking
- Certain medications, such as allopurinol (Zyloprim), hydralazine (Apresoline), minocycline (Dynacin, Minocin, Myrac, Solodyn, Ximino), and propylthiouracil
- COVID-19, hepatitis A, or hepatitis B infections
- Also having other immune disorders
- Your sex. Certain types are more likely to affect people of a particular gender.
How common is it?
Most types of vasculitis are very rare. Fewer than 50 out of 1 million people get vasculitis every year in the U.S.
You're more likely to get it if you're over 50. But your odds are still very low. Only about 300 out of 1 million people older than 50 in the U.S. are diagnosed annually.
Vasculitis Diagnosis
Your doctor will ask about your medical history and do a physical exam. There's no test just for vasculitis. But because it tends to result from other conditions, you may need tests to look for inflammation and figure out what's causing your symptoms. These tests may include:
- Blood tests. Certain types of blood cells or antibodies can be signs of vasculitis.
- Urine tests. These check for kidney damage.
- Imaging tests. X-rays, MRI scans, CT scans, PET scans, and ultrasounds show inflammation in your blood vessels and organs. You might also have an angiogram, in which your doctor injects dye into your bloodstream. It shows up on X-rays to give a better picture of your blood vessels.
- Heart tests. An echocardiogram tests how well your heart is working.
- Biopsy. Your doctor takes a sample of tissue. A specialist can check it for signs of inflammation or damage.
Vasculitis Treatment
Which vasculitis treatment your doctor recommends depends on what’s causing it and which organs it affects. It's usually meant to control the inflammation and prevent organ and blood vessel damage.
Medications
Steroids like prednisone are the most common medications prescribed to fight the inflammation vasculitis causes. Your doctor will watch you closely for side effects like high blood pressure, high blood sugar, and bone problems, especially if you take them for a long time.
Other medications, like azathioprine (Azasan, Imuran), cyclophosphamide (Cytoxan), methotrexate (Rheumatrex, Trexall), mycophenolate (CellCept, Myfortic), rituximab (Riabni, Rituxan, Ruxience, Truxima), or tocilizumab (Actemra) can be prescribed along with steroids. Which medication you might need depends on how serious your vasculitis is, whether it's in your organs, and your medical history.
Surgery
Sometimes vasculitis can cause issues that need surgery to repair. For instance, if your blood vessel walls bulge and form an aneurysm, surgery can lower the chances that it will burst. If you have a blocked artery, you could need surgery to restore blood flow to the area. But any kind of organ damage might require surgery.
Vasculitis Complications
Whether you have complications depends on what type of vasculitis you have and how bad it is. Some serious complications of vasculitis include:
- Organ damage
- Blood clots
- Aneurysm
- Loss of eyesight
- Infection
Vasculitis Prognosis
There's no cure for vasculitis, but with the right treatment, you can live a long and active life. Most types of vasculitis are lifelong. But successful treatment can give you long periods without symptoms (called remissions).
Your outlook depends on several things, including:
- The type of vasculitis you have
- How quickly you were diagnosed
- Which organs are affected and how seriously
- Other health problems you have
Living with vasculitis
For many people, the hardest part about vasculitis is managing the side effects of medications. There are steps you can take to manage these and other day-to-day issues:
Learn and understand the disease. Most types of vasculitis have periods of remission and relapse. Stick to your treatment plan and let your doctor know about any new symptoms or health changes.
Exercise regularly. Not only can exercise boost your mood and lower stress, it can help parts of your body that your treatment affects. Regular walking, for instance, can reduce your chances of bone loss, high blood pressure, and diabetes caused by corticosteroids.
Adopt healthy food habits. Focus on fresh fruits and vegetables, whole grains, low-fat dairy products, lean meat, and fish. And limit alcohol, sugar, and fat. A healthful diet can help with medication side effects like thinning bones, high blood pressure, and high blood sugar. If you take corticosteroids, ask your doctors about calcium and vitamin D supplements.
Keep your vaccinations updated to help prevent infections, like pneumonia and the flu, that can stem from your medications.
Surround yourself with support, whether it's from family, friends, or a support group. Your health care team can also refer you to a mental health professional.
Takeaways
Vasculitis is inflammation of your blood vessels. It thickens your blood vessels, sometimes so much that blood can't flow properly. This can damage your organs and tissues. It's a lifelong disease without a cure, but it can be treated, usually with steroids.
Vasculitis FAQs
Is vasculitis very serious?
Many types of vasculitis can be serious, specifically when they restrict blood flow. This not only can damage your organs and cause serious issues like aneurysms, but it could also be fatal. Certain kinds of vasculitis can cause vision loss or blindness, if they're not treated.
What does vasculitis look like when it starts?
If your vasculitis has symptoms you can see on your body, it'll likely appear as a rash, or spots of red, purplish red, black, or simply discolored skin. A vasculitis rash can be on your fingers, legs, ankles, or toes. Other signs that can point to vasculitis are swelling in your joints or cramps and bloating.