What Is Vasculitis?

Medically reviewed by Stella Bard, MD

Vasculitis is a rare autoimmune disorder that causes inflammation of your blood vessels (such as the arteries, capillaries, and veins). The condition develops when your immune system accidentally attacks the blood vessels, sometimes due to an underlying condition, infection, or medication.

With vasculitis, the inflamed blood vessels narrow and restrict blood flow to the body's organs and tissues. Depending on the type of blood vessel involved, vasculitis can affect everything from your skin and joints to your gastrointestinal tract and internal organs.

If you have symptoms of vasculitis, see your healthcare provider as soon as possible. If left untreated, this condition can cause life-threatening complications. Treatment options for vasculitis vary and will depend on the type of vasculitis you have. In some cases, certain forms of vasculitis may improve over time. But, most types of vasculitis cause symptoms that may require lifelong management to prevent complications and improve your quality of life.

Types of Vasculitis

Knowing the type of vasculitis you have helps healthcare providers treat the condition effectively. There are several forms of vasculitis, which are classified based on the size and location of the blood vessels that the condition is affecting. These include:

Symptoms

Symptoms of vasculitis vary from person to person, depending on the type of vasculitis and which organs and blood vessels are affected. For some people, symptoms develop slowly over months, while others experience a more sudden onset of symptoms that develop over days or weeks. For most people, vasculitis symptoms tend to come and go. 

Systemic Symptoms

Vasculitis is a systemic disorder—meaning that it can affect blood vessels anywhere in the body. Some people with vasculitis develop general symptoms, such as:

• Fatigue

• Fever

• Body-wide aches and pains

• Loss of appetite

• Weight loss

Organ-Specific Symptoms

Depending on the type of vasculitis you have, you may experience symptoms related to the blood vessels in a specific organ or body part, such as:

• Skin: Purple or red spots or bumps, small dots, hives, bruises, and ulcers

• Joints and muscles: Pain, stiffness, weakness, and swelling

• Nerves: Numbness or tingling sensations and loss of feeling in hands and feet

• Lungs: Shortness of breath, coughing up blood-tinged mucus, and chest pain

• Gastrointestinal tract: Abdominal pain and mouth sores

• Eyes: Redness, burning, itchy sensation, blurred vision, and light sensitivity

• Sinuses, ears, nose, and throat: Nasal congestion, chronic middle ear infections, or sores in the nose

• Brain: Headaches, changes in mental awareness (e.g., confusion), or stroke-like symptoms such as limb paralysis or weakness

Causes and Risk Factors

Vasculitis occurs when your body's immune system mistakenly perceives your blood vessels as foreign invaders and launches an attack. This can lead to inflammation in your blood vessels, which makes your vessels become narrow and weak. Ultimately, this response can block blood flow to your body's organs and tissues.

Exactly what triggers this immune response is not always clear. But, researchers suspect that several factors likely contribute to the development of vasculitis. Possible triggers that can increase your risk of vasculitis include:

• Age: Some types of vasculitis are more common in young children (e.g., IgA vasculitis, Kawasaki disease), while other types are more common in older adults (e.g., giant cell arteritis)

• Family history: If someone in your family also has a certain type of vasculitis (e.g., Kawasaki disease), you may also have a higher chance of developing the condition

• Infections: Certain infections, such as hepatitis B and hepatitis C, can trigger vasculitis

• Medications: Some medications, such as hydralazine (to treat high blood pressure) or Tumor necrosis factor inhibitors (to treat some autoimmune diseases), are associated with an increased risk of vasculitis

• Environmental factors: Some experts suggest that exposure to toxic chemicals and experiencing stress may trigger an immune response

• Other autoimmune diseases: People with other autoimmune diseases, such as lupus and rheumatoid arthritis, are at increased risk of developing vasculitis

• Lifestyle habits: Smoking tobacco and using other drugs can sometimes lead to an immune response that causes vasculitis
  

Diagnosis

Healthcare providers diagnose vasculitis by performing a physical examination, reviewing your medical history, and ordering diagnostic tests. Your healthcare provider will determine which diagnostic tests to order based on your symptoms. These tests help diagnose vasculitis and identify the underlying cause, if unknown. 

Common diagnostic tests for vasculitis include:

• Blood tests: Measures your blood cell count (e.g., how many red and white blood cells you have) and checks for markers of inflammation and infection

• Biopsy: Removes a small tissue sample from the affected organ or blood vessel to view it under a microscope and check for signs of tissue damage

• Chest X-ray: Provides images of your chest cavity to determine whether your lungs or large arteries (e.g., aorta) are affected

• Computed tomography (CT) scan: Takes detailed images of your internal organs to look for signs of blood vessel damage

• Echocardiography (ultrasound of your heart): Helps visualize how well your heart is functioning

• Coronary angiography: Involves contrast dye (e.g., iodine) and X-ray pictures of your chest to check for signs of damage, inflammation, or blood clots in the arteries that wrap around your heart (coronary arteries)

• Ultrasound: Looks for signs of damage to your blood vessels or internal organs

• Positron electron tomography (PET) scan: Uses a small amount of radioactive material to visualize the blood vessels and check for internal inflammation and damage

• Urinalysis: Requires a urine sample to assess kidney function and determine whether the condition is affecting your kidneys

Treatment

The goal of treatment for vasculitis is to reduce inflammation in the blood vessels to achieve remission—or, a period in which you have no symptoms. Early diagnosis and treatment can help prevent damage to organs and tissues. 

Treatments vary based on the type of vasculitis you have and the severity of your symptoms. Over-the-counter anti-inflammatory medications, such as Advil (ibuprofen) or Aleve (naproxen), may be enough to reduce pain and discomfort for mild vasculitis. More severe vasculitis may require prescription medications or specialist procedures.

Corticosteroids

Corticosteroids are anti-inflammatory drugs that help reduce inflammation. Prednisone and other corticosteroids are usually the first-line treatment for vasculitis. Depending on the type and severity of vasculitis, you may need corticosteroids for months or years. These medicines are generally well-tolerated but may increase blood sugar or blood pressure and reduce bone density.

Immunosuppresants

Medications that suppress the immune system treat more severe forms of vasculitis or when corticosteroids are ineffective at reducing inflammation. In some cases, healthcare providers may prescribe corticosteroids along with an immunosuppressant medicine, such as:

• Trexall or Rheumatrex (methotrexate)

• Imurine (azathioprine)

• Cytoxin (cyclophosphamide)

• Cellcept or Myforin (mycophenolate mofetil)
  

Biologics

Biologic drugs target specific parts of the immune system involved in inflammation. These medicines treat specific types of vasculitis, such as giant cell arteritis and eosinophilic granulomatosis with polyangiitis (EGPA). Biologics can reduce inflammation and often have fewer side effects than other medicines. Your healthcare provider may prescribe:

• Rituxan (rituximab)

• Actemra (tocilizumab)

• Nucala (mepolizumab)

Specialist Procedures

If blood flow is severely restricted and harming organs and body tissues, specialist procedures can help improve blood flow. These treatments include:

• Plasmapheresis: A procedure that involves filtering plasma (the liquid portion of your blood) to remove harmful antibodies and other inflammatory substances from the bloodstream

• Angioplasty: A minimally invasive procedure that uses a medical balloon to widen narrowed or blocked blood vessels to improve blood flow to the affected organs and tissues

• Bypass surgery: A surgical procedure that allows your surgeons to create new pathways for blood to flow by using a healthy blood vessel from another part of the body or using synthetic material to improve blood flow to the affected area

How to Prevent Vasculitis Flares

Like most autoimmune conditions, there is no surefire way to prevent vasculitis. However, following your treatment plan, seeing your healthcare provider regularly, and adopting healthy lifestyle habits can help control inflammation and reduce the frequency and severity of symptom flare-ups. The following may help prevent vasculitis flares:

• Eat a nutritious, balanced diet

• Participate in exercise or physical activity daily

• Get plenty of sleep

• Manage stress through activities such as yoga, meditation, or breathwork

• Quit smoking

• Avoid excess alcohol consumption

• Stay up-to-date on routine vaccinations, including COVID-19 and the flu

Related Conditions

Vasculitis is associated with several related conditions, either due to shared underlying factors or the effects of vasculitis on your vital organs. The most common related conditions of vasculitis are:

• Rheumatoid arthritis: An autoimmune disease that causes joint inflammation and pain. Some forms of vasculitis, such as rheumatoid vasculitis, can occur alongside rheumatoid arthritis, affecting blood vessels and joints.

• Systemic lupus erythematosus (lupus): An autoimmune disorder that often causes blood vessel inflammation (vasculitis).

• Scleroderma: Another type of autoimmune disease that can cause thickened skin, joint pain, gastrointestinal problems, and inflamed blood vessels.
  

Untreated vasculitis can also lead to serious complications, such as:

• Heart disease

• Stroke

• Aneurysm

• Kidney disease

• Nerve damage

• Vision problems

Living With Vasculitis

With early diagnosis and treatment, many people with vasculitis can achieve remission and experience long periods of symptom relief. Flares are possible with most types of vasculitis, so being proactive about your health by following your treatment plan and working closely with your healthcare provider is essential to living well. 

The outlook for people with vasculitis depends on the type and severity and how early the condition was diagnosed and treated. Most people with vasculitis who follow their treatment plan can live fulfilling, productive lives. However, some types of vasculitis can lead to serious complications, such as organ damage, heart disease, and stroke.

Living with vasculitis means there may be times you feel exhausted or overwhelmed by your symptoms or medication side effects. Be patient, rest when needed, and lean on family and friends for support. It may be helpful to join a vasculitis support group to connect with others who share similar experiences.

Frequently Asked Questions

What does vasculitis look like when it starts?

Vasculitis can look different depending on the type and severity of the disease. It often begins with non-specific symptoms like fever, fatigue, muscle aches, joint pain, and weight loss. As it progresses, you may develop additional symptoms depending on which blood vessels are affected.

What parts of the body does vasculitis affect?

Vasculitis can affect virtually any body part, including the skin, joints, muscles, nerves, eyes, sinuses, lungs, gastrointestinal tract, kidneys, and the brain. The specific organs and systems involved vary depending on which blood vessels are involved.

Is vasculitis an autoimmune disorder?

Yes, vasculitis is an autoimmune disorder. It occurs when the immune system mistakenly attacks blood vessel walls, leading to inflammation. The exact cause of the abnormal immune response is not always clear but is often the result of infections, certain medications, or underlying conditions.

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