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Vasculitis Causes, Symptoms, and Treatments

Vasculitis means inflammation of the blood vessels.

Vasculitis causes a wide range of symptoms, such as:

  • Fatigue.
  • Fever.
  • Vasculitis rashes.

Symptoms can vary widely based on where the swelling happens in the body. Treatments for vasculitis reduce swelling and relieve symptoms.

Learn more about:

  • Vasculitis symptoms.
  • Vasculitis diagnosis.
  • Vasculitis treatment.

Looking for Vasculitis Care?

  • Vasculitis Services at UPMC.

Contact UPMC Rheumatology and Clinical Immunology

Call 1-888-349-1506 to make an appointment at a UPMC rheumatology location near you.

Find a Vasculitis Provider

Request an Appointment


What Is Vasculitis?

Vasculitis is when the immune system attacks the blood vessels. Doctors define it as an umbrella of health issues that cause inflammation in the blood vessels.

The swelling can lead to scarred, narrowed, and weakened blood vessels.

Vasculitis may affect one or many parts of the body.

Is vasculitis a serious disease?

It can be.

Without treatment, some types of vasculitis can lead to:

  • Heart attacks.
  • Organ damage, such as renal failure.
  • Strokes.
  • Death.

What Are the Different Types of Vasculitis?

There are more than 15 types of vasculitis.

Some types affect mostly older people. Others are more common in children.

Doctors often classify vasculitides (the plural form of vasculitis) by the size of the blood vessel involved.

Large-vessel vasculitis

  • Aortitis.
  • Giant cell arteritis (and polymyalgia rheumatica).
  • Takayasu's arteritis.

Medium-vessel vasculitis

  • Kawasaki disease.
  • Polyarteritis nodosa.

Small-vessel vasculitis

Anti-neutrophil cytoplasmic antibody (ANCA) vasculitis, which includes:

  • Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis).
  • Microscopic polyangiitis.
  • Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome).
  • Cryoglobulinemic vasculitis.
  • IgA vasculitis/Henoch-Schönlein purpura.
  • Drug-induced vasculitis or hypersensitivity vasculitis.
  • Primary angiitis of the central nervous system.

Vasculitis that affects blood vessels of different sizes

  • Behcet's disease/syndrome.
  • Relapsing polychondritis.

Vasculitis linked to other rheumatic diseases

  • Rheumatoid vasculitis.
  • Sjogren's disease.
  • Systemic lupus erythematosus.

What Are the Risk Factors and Complications of Vasculitis?

Vasculitis risk factors

The following risk factors can lead to changes in the immune system or blood vessels that make vasculitis more likely:

  • Autoimmune disorders, like lupus or rheumatoid arthritis.
  • Certain medicines, such as hydralazine, levamisole, and tumor necrosis factor inhibitors, can lead to drug-induced vasculitis.
  • Cocaine use.
  • Genetics — some forms of vasculitis more commonly run in families.
  • Lymphoma, which is a type of blood cancer.
  • Smoking.
  • Viral, fungal, or bacterial infections. Hepatitis B and C are the most common triggers.

Complications of vasculitis

Based on the type and how severe it is, vasculitis can lead to:

  • Blood clots in the lungs and/or legs.
  • An aneurysm — a bulge in a blood vessel that can burst and be deadly.
  • Heart attacks.
  • Organ damage.
  • Stroke.

Treatments and routine check-ups help prevent these problems.

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What Are the Signs and Symptoms of Vasculitis?

People who have vasculitis generally don't feel well.

They may have:

  • Chills.
  • Fatigue.
  • Fever.
  • Joint pain.
  • Loss of appetite.
  • Weight loss.

Vasculitis symptoms depend on the type you have, so they can vary widely. The symptoms also depend on where in the body the swelling is.

Symptoms of vasculitis can occur in the:

  • Brain and spine — headaches, vision changes, memory loss, numbness, or weakness.
  • Ears, nose, and throat — congestion, ear pain, hearing impairments, nose bleeds, or voice changes.
  • Eyes — blood vessel swelling that can lead to vision changes or loss and red eyes.
  • Digestive system — abdominal pain or blood in the stool.
  • Legs, arms, hands, and feet — swelling and weakness.
  • Lungs — shortness of breath and coughing. Some may cough up blood.
  • Skin — a dark red rash or small bruises.

What does vasculitis look like when it starts?

Many types of vasculitis may look like nothing at all. Most of the symptoms aren't visible.

The symptoms that you may see vary based on:

  • The type of vasculitis.
  • Where it is in the body.
  • Whether it's severe or mild.

Some visible symptoms include a dark red rash or small bruises if vasculitis is in the skin. The rash shows up as red skin welts and raised hives. These can itch and burn.

Skin patches of vasculitis have red rims and white centers. They may have red or purple pinpoint spots caused by bleeding under the skin (petechia).

Other visible symptoms include red eyes and swelling in the legs, arms, hands, or feet.

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How Do You Diagnose Vasculitis?

Vasculitis symptoms are also common in many other health issues. For this reason, it may take time to rule out other diseases before diagnosing vasculitis.

Your doctor will do an exam and check your heart rate and blood pressure. You will often need more than one test to confirm vasculitis.

These tests may include:

  • Blood tests to check for antibodies present in some forms of the disease.
  • Urine tests to check for high levels of certain proteins, which can mean vasculitis in the kidney.
  • Imaging tests — such as x-rays, CT scans, MRIs, PET scans, ultrasound, and angiography — to look for swelling. You may need to take a dye for the test.
  • A biopsy to take a small tissue sample. Your doctor will then send it to a lab to check for signs of inflammation in the blood vessels.

Other tests can show if vasculitis is affecting you.

Your doctor may suggest:

  • A lung function test to check the lungs.
  • A laryngoscopy where they use a camera through a tube to check for signs of throat problems.
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What Are the Treatment Options for Vasculitis?

Corticosteroids

The most common treatment for vasculitis is corticosteroids, medicines that reduce swelling.

As these drugs are unsafe for long-term, high-dose use, your doctor will prescribe them sparingly. After the disease is under control, they'll reduce or stop the medicine.

Immune-suppressing medicines

Your doctor may also suggest other drugs that block certain immune proteins.

These include medicines that target B-cells, a type of white blood cell that triggers inflammation.

Biologic drugs

Biologics are a newer type of treatment made from complex sources, like cells.

As many of them target a specific process in the body, they can have fewer side effects than other treatments.

Surgery

People may need surgery if vasculitis causes a bulging blood vessel or blocked blood clot.

Other vasculitis treatments

You may need other treatments, depending on how vasculitis affects you.

If it affects your lungs, you may need an inhaler to help you breathe. If you get skin rashes, you may need lotions.

Physical therapy can also help people if vasculitis or treatments affect their strength or movement.

How Long Can a Person Live with Vasculitis?

While it can be fatal, many people with vasculitis live into old age.

The outlook depends on many factors, including the type of vasculitis, treatments, lifestyle, and more.

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