Vasculitis is a group of diseases, also known as angiitis, that damage blood vessels by causing inflammation. It can affect any blood vessel anywhere in the body.

Vasculitis means inflammation of the blood vessels. It can affect different body parts, and the impact and symptoms will depend on the affected area. It causes alterations in the walls of blood vessels, which may include scarring, weakening, narrowing, and thickening.

Vasculitis can be acute and short-term or chronic and long-term. In some cases, the organs in the body may be affected, especially if they do not receive enough nutrient and oxygen-rich blood, resulting in organ damage and sometimes death.

This article covers the types, symptoms, causes, and treatments of vasculitis.

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There are two main categories of vasculitis. Primary vasculitis, which is vasculitis with no known cause, and secondary vasculitis, which occurs because of another disease such as:

  • Infection: Hepatitis C virus infection can cause cryoglobulinemia.
  • Immune system disorder: Rheumatoid arthritis and lupus are examples.
  • Allergic reaction: Some medications can cause vasculitis.
  • Some cancers: Leukemia and lymphoma are examples.

Specific types

These affect different parts of the body and will result in a wide variety of symptoms.

Signs and symptoms of vasculitis vary depending on which blood vessels are affected and which organs are damaged, if any.

Most cases of vasculitis include the following symptoms:

  • fever
  • tiredness
  • weight loss, loss of appetite
  • joint pain
  • muscle pain
  • numbness and weakness

The following are signs and symptoms of specific vasculitis conditions:

Behcet’s disease

Behcet’s disease may cause the following symptoms depending on the affected area.

  • lesions in the mouth, genitals, or on the skin
  • inflammation in the eye (uveitis)
  • joint swelling
  • inflammation in the veins and large arteries
  • abdominal pain, diarrhea, and digestive bleeding
  • inflammation of the central nervous system, leading to headaches and disorientation

Buerger’s disease

Buerger’s disease affects the small and medium blood vessels in the arms and legs. The blood vessels swell and become blocked with blood clots, called thrombi, eventually damaging or destroying skin tissues. Sometimes, this can lead to infection and gangrene.

Initial symptoms often include pain in the feet and hands during exercise caused by insufficient blood flow. A person may also experience:

  • numbness in the limbs
  • tingling in the limbs
  • fingers, toes, hands, and feet turn white in the cold
  • skin ulcerations
  • gangrene of fingers and toes
  • affected areas may be extremely painful

Central nervous system vasculitis

This is vasculitis that involves the brain and spinal cord. Signs and symptoms may include:

  • mental changes
  • headaches
  • general confusion
  • paralysis or muscle weakness
  • visual problems
  • dysphasia, coma
  • seizures
  • depression
  • myelopathy, which is a disorder of the spinal cord

Churg-Strauss syndrome

This rare syndrome is an inflammation of small arteries and veins that is associated with asthma, rhinosinusitis, and increased eosinophils, a type of white blood cells. Early signs and symptoms may include:

In the next phase, the person typically has eosinophilia, a condition when there are too many eosinophils, a type of white blood cell.

The third phase is vasculitis, usually involving the skin, lungs, nerves, kidneys, and other organs. There is frequent devastation of the nerves, called mononeuritis multiplex, which causes severe tingling, numbness, muscle wasting in hands and feet, and shooting pains.

Cryoglobulinemia

This condition causes the blood to become abnormally thick with inflammation of blood vessels.

Symptoms vary according to the type and which organs are affected. Typically, signs and symptoms include:

  • breathing problems
  • fatigue
  • glomerulonephritis affecting the kidneys
  • joint pain
  • muscle pain
  • purpura, or purple spots and patches on the skin, organs, and mucous membranes
  • fingers, toes, hands, and feet turning white in the cold
  • skin ulceration

Giant cell arteritis

This is inflammation of the walls of medium to large arteries in persons over 50. The most common symptoms are:

  • headache
  • shoulder pain
  • pain in the hips
  • pain in the jaw after chewing
  • fever
  • blurred vision

Henoch-Schönlein purpura

Inflammation of the tiny blood vessels, called the capillaries, in the skin and frequently in the kidneys, resulting in skin rashes, especially over the buttocks and behind the lower extremities.

Other symptoms include:

  • joint pain, especially in the knees and ankles
  • stomach pain
  • nausea
  • vomiting
  • diarrhea
  • blood in stools if the vessels in the bowel and kidneys are inflamed

Kawasaki disease

This is a rare syndrome that causes inflammation in the walls of arteries throughout the body, including the coronary arteries supplying blood to the heart muscle.

Kawasaki disease causes high fever, reddening of the eyes (conjunctivitis), lips and mucous membrane of the mouth, gingivitis (ulcerative gum disease), swollen neck glands, and a bright red rash on the hands and feet. It affects young children.

Takayasu’s arteritis

This is a rare type of vasculitis in which inflammation damages the aorta, the large artery carrying blood from the heart to the rest of the body. It also damages the aorta’s main branches.

A person with Takayasu’s arteritis experiences arm or chest pain, hypertension causing high blood pressure, and they may eventually experience a stroke or heart failure.

Wegener’s granulomatosis

Wegener’s granulomatosis causes inflammation and injury to blood vessels and affects several organs, including the lungs, kidneys, and upper respiratory tract.

It is a life threatening disorder that requires long-term immunosuppression. Some people can die due to the toxicity of the treatment.

Rapidly progressive glomerulonephritis

This condition is a syndrome of the kidney that rapidly progresses into acute renal (kidney) failure if untreated.

Vasculitis occurs when the body’s immune system mistakenly attacks its blood vessels. Some known triggers are:

  • infections
  • some cancers
  • some immune system disorders
  • allergic reactions

Typically, when blood vessels are affected by vasculitis, they become inflamed. This causes:

  • the blood vessel walls to thicken
  • the blood vessels to narrow
  • the blood flow to be reduced

Less blood flow means less oxygen and nutrients reaching organs and body tissues. As a result, the affected blood vessel is more susceptible to blood clots, partly because it is narrower. If the blood vessels weaken, an aneurysm, or bulge, may form.

The primary care doctor or specialist will ask someone about their symptoms and medical history and perform a physical examination. The following diagnostic tests may be ordered as well:

Blood tests

These may include:

  • Erythrocyte sedimentation rate (ESR) test: A sample of red blood cells is put into a test tube of liquid. The time the red blood cells fall to the bottom is measured. If they fall faster than normal it could mean an inflammatory condition.
  • C-reactive protein (CRP) test: A higher than usual blood level of CRP indicates an inflammation in the body.
  • Complete blood cell count: This measures red blood cells white blood cells, and platelets
  • Chemistry: This includes electrolytes, kidney function, and liver function.
  • Antibody tests: Doctors use these to determine presence of specific antibodies that are suspected as the cause.

Other tests

Other tests a doctor may order include

Treatment for vasculitis depends on several factors, including what type of vasculitis the person has, the severity of their symptoms, age, and general health.

Steroids

These are used to reduce inflammation. Examples include prednisone or methylprednisolone (Medrol).

Immune system medications

If a person does not respond well to steroid therapy, a doctor may prescribe medications that decrease the activity of immune system cells. Examples include:

  • azathioprine (Imuran)
  • cyclophosphamide (Cytoxan)

Vasculitis refers to a range of rare conditions where inflammation damages blood vessels. It can affect any type of blood vessel, anywhere in the body.

The symptoms and treatments for vasculitis will vary depending on its severity and location.