Autoimmune vasculitis is a group of conditions that cause blood vessel inflammation. Examples include Kawasaki disease and giant cell arteritis. They develop as a result of an overreaction of your immune system.

Researchers have identified many types of autoimmune vasculitis. Some of these conditions, such as Kawasaki’s disease, occur primarily in children. Others, such as eosinophilic granulomatosis with polyangiitis, tend to develop in adults.

Symptoms of autoimmune vasculitis can vary depending on the type of condition and which parts of your body it affects. Treatment can also depend on the type of autoimmune vasculitis you have.

Read on to learn more about autoimmune vasculitis, including the types, symptoms, causes, and more.

Autoimmune vasculitis can be classified as small, medium, or large depending on the size of the affected blood vessels.

Types of autoimmune vasculitis that researchers have identified include:

  • Giant cell arteritis: This condition mostly affects the aorta, the main blood vessel that carries blood away from your heart, and its major branches. It often causes loss of blood flow through the temporal artery, which supplies blood to your scalp and face.
  • Kawasaki disease: Kawasaki disease is a rare childhood condition that affects blood vessels throughout the body and often affects the coronary arteries.
  • Microscopic polyangiitis (MPA): MPA affects small or medium blood vessels in your body. It most commonly affects those in your kidneys or lungs.
  • Granulomatosis with polyangiitis (GPA): GPA usually affects blood vessels in your nose, throat, lungs, and kidneys. It also affects your sinuses.
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): This condition generally affects your respiratory tract, gastrointestinal tract, skin, heart, and nervous system.
  • Behçet’s disease: Behçet’s disease, also called Behçet’s syndrome, can affect many parts of your body, including your genitals, skin, eyes, and mouth.

Non-autoimmune causes of vasculitis

Vasculitis is not always caused by autoimmune conditions. Possible non-autoimmune causes include:

Symptoms of autoimmune vasculitis can vary depending on which type of vasculitis you have.

Giant cell arteritis symptoms

Giant cell arteritis can cause symptoms such as:

Kawasaki disease symptoms

Kawasaki disease may cause symptoms such as:

  • high fever that lasts at least 5 days
  • swollen feet and hands
  • a rash, most commonly affecting your groin, chest, or back
  • pink eye (conjunctivitis)
  • swollen lymph nodes in your neck
  • redness or discoloration of your:
    • lips
    • tongue
    • mouth lining
    • palms of your hands
    • soles of your feet

MPA symptoms

Some MPA symptoms will depend on which organs the condition affects.

Common general symptoms can include:

  • tiredness
  • joint aches
  • muscle aches
  • loss of appetite

GPA symptoms

General symptoms of GPA are usually mild and can include:

  • high temperature
  • weakness
  • tiredness
  • night sweats
  • joint pain
  • loss of appetite
  • unexplained weight loss

Other symptoms can depend on which parts of your body the condition affects.

Eosinophilic granulomatosis with polyangiitis symptoms

Eosinophilic granulomatosis with polyangiitis commonly causes:

  • a tendency to feel tired and unwell
  • loss of appetite
  • asthma
  • sinus polyps
  • fever
  • an increased level of a type of white blood cells called eosinophils

Other symptoms will depend on which organs the condition affects but may include:

  • shortness of breath
  • chest pain
  • coughing
  • numbness or weakness
  • tingling in your hands and feet
  • muscle pain
  • joint pain
  • facial pain
  • nasal discharge
  • abdominal pain
  • bloody stool
  • rash
  • kidney disease

Behçet’s disease symptoms

Behçet’s disease can commonly cause symptoms such as:

  • genital and mouth ulcers
  • joint pain
  • headaches
  • stomach pain
  • tiredness

Other symptoms may also develop since the condition can affect most parts of your body.

The underlying cause of vasculitis is often unclear. However, medical professionals have identified factors that may increase the likelihood of developing each condition:

  • Giant cell arteritis: This condition occurs almost exclusively in people older than 50 years. It most commonly occurs in the late 60s and 70s. People of Northern European ancestry seem to be at the highest risk.
  • Kawasaki disease: Most people who have Kawasaki disease are under 5 years old. It is rare in children over 8 years old.
  • MPA: MPA may have a genetic component, but more research is necessary. Exposure to infections or toxins may trigger this condition.
  • GPA: An infection may increase your likelihood of developing GPA. It can occur at any age but is more common in people between 40 and 65 years old.
  • Eosinophilic granulomatosis with polyangiitis: The cause of eosinophilic granulomatosis with polyangiitis is unclear. It can develop at any age but is more likely to occur between the ages of 35 and 50 years.
  • Behçet’s disease: Behçet’s disease symptoms tend to begin in early adulthood. It’s thought that people of Mediterranean, Asian, and Middle Eastern origin are at the highest risk.

Receiving a diagnosis of autoimmune vasculitis often involves working with a team of medical experts, such as:

  • rheumatologists
  • cardiologists
  • neurologists

If doctors suspect autoimmune vasculitis, they may order numerous tests, including:

  • blood tests
  • urine tests
  • MRI
  • X-rays
  • PET scan
  • biopsy

If you need any tests, a doctor can provide more information about what they involve and answer any questions you may have.

Treatment for autoimmune vasculitis usually focuses on medications to reduce the activity of your immune system. The type of treatment you receive may depend on the type of the condition you have but can include:

  • over-the-counter pain relief medication (in milder cases)
  • corticosteroids
  • anti-inflammatory medications, such as nonsteroidal anti-inflammatory drugs (NSAIDs)
  • immunomodulators
  • immunosuppressive medications
  • dual endothelin receptor antagonists
  • interleukin antagonists
  • interferon therapy
  • monoclonal antibodies
  • phosphodiesterase inhibitors
  • tumor necrosis factor inhibitors
  • intravenous immunoglobulin (IVIg)

After diagnosis, a doctor can help create a suitable treatment plan based on the type of autoimmune vasculitis you have and your personal circumstances.

Many complications are possible, depending on the type of vasculitis you have.

For example, about 1 in 4 children with Kawasaki disease who do not receive treatment develop heart-related complications. These complications can be fatal in about 2% to 3% of cases.

Examples of other complications that can develop as a result of vasculitis or its treatment include:

If you receive a diagnosis of autoimmune vasculitis, your doctor can provide more information about the possible complications and any steps you can take to manage them or reduce your chances of experiencing them.

Autoimmune vasculitis is a group of conditions that cause inflammation in your blood vessels. These conditions can develop if your immune system mistakenly attacks healthy tissue in your blood vessels.

Types of autoimmune vasculitis include giant cell arteritis, Kawasaki disease, microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), and Behçet’s disease.

Treatment for autoimmune vasculitis often consists of corticosteroids and other medications to reduce immune system activity. If you have autoimmune vasculitis, you can work with your doctor to create a suitable treatment plan and manage your risk of complications.