Vasculitis refers to a large group of diseases that cause inflammation of the blood vessels.

The specific blood vessels affected include arteries, veins, and the tiniest of these known as capillaries. It can affect different parts of the body, leading to a wide range of symptoms.

Fast facts on vasculitis

  • Vasculitis can have a large number of different forms.
  • Symptoms of vasculitis can include fever, tiredness, and joint pain.
  • Central nervous system vasculitis can cause mental changes and seizures.
  • Vasculitis has a number of potential causes including infections and immunologic diseases.
  • Treatment for vasculitis often includes steroids.

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Vasculitis can damage specific organs by reducing oxygen supply.

Vasculitis means and inflammation of the blood vessels. It can affect different parts of the body, and the impact and symptoms will depend on which part is affected.

Vasculitis is also called angiitis and arteritis. 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.

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

Sometimes, as may be the case with Henoch-Schonlein purpura, the condition clears up without medical treatment.

Listed here are some of the most commonly used medications for vasculitis:

Steroids

These are used to reduce inflammation. Examples include prednisone or methylprednisolone (Medrol). Side effects may be severe if taken over the long-term. They may include:

Immune system medications

If a person does not respond well to steroid therapy, a doctor may prescribe cytotoxic drugs that stop the immune system cells that cause inflammation.

Examples include:

  • azathioprine (Imuran)
  • cyclophosphamide (Cytoxan)

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

Symptoms vary and can disappear and return. The affected areas include:

  • Mouth: Painful sores, like canker sores. They start as raised, round lesions in the mouth and become painful ulcers.
  • Skin: Some people may develop acne-like sores while others may have red, raised, and tender nodules, especially on the legs.
  • Genitals: Most commonly on the scrotum in males and the vulva in females. They appear as red, round, ulcerated lesions.
  • Eyes: Inflammation of the eye, called uveitis.
  • Joints: Typically swelling and pain in the knees, and sometimes the ankles, elbows, or wrists.
  • Blood vessels: Inflammation of the veins and large arteries causing painful and swollen limbs.
  • Gastrointestinal: Abdominal pain, diarrhea. Possible bleeding in the digestive system.
  • Brain: Inflammation in the brain and nervous system can result in headaches, fever, poor balance, and disorientation.

Buerger's disease

Buerger's disease affects the arteries and veins 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. Risk of Buerger's disease is linked to regular smoking.

Initial symptoms often include pain in the feet and hands during exercise caused by insufficient blood flow. Sometimes this pain may also be present at rest. Usually the pain starts in the extremities but may radiate to other parts of the body. 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

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Central nervous system vasculitis can induce mental changes.

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 in people with a history of allergy or asthma.

Its main feature is asthma, which may begin long before the onset of vasculitis. 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 a 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 is linked to hepatitis C infections. It 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 the arteries. The most common symptoms are:

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

Less common symptoms include:

  • scalp tenderness
  • cough
  • throat pain
  • tongue pain
  • weight loss
  • depression
  • pain in the arms during exercise

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. It is associated with arthritis and sometimes cramping pain in the tummy.

The most common symptoms are:

  • a skin rash that looks similar to small bruises, or red spots on the buttocks, elbows, and legs
  • 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 of unknown origin. It 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 where inflammation damages the aorta, which is 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

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One of the symptoms of Wegener's granulomatosis is eye pain.

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.

Complications

Complications can vary depending on the type of vasculitis. The following are the most common complications:

  • Organ damage: Poor oxygen and nutritional blood supply to organs can lead to damage.
  • Recurrence: Even if treatment is successful there may be recurrent episodes. Some people require long-term treatment.

Warning: These images are graphic:

Vasculitis occurs when the body's immune system mistakenly attacks its blood vessels. Experts are not sure why this happens. 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 getting to 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.

There are two main categories of vasculitis:

Primary vasculitis, which is vasculitis with no known cause, and secondary vasculitis, which occurred 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.

Some specific types of vasculitis are:

  • Behcet's disease
  • Buerger's disease
  • central nervous system (CNS) vasculitis
  • Churg-Strauss syndrome
  • cryoglobulinemia
  • giant cell arteritis
  • Henoch-Schönlein purpura
  • Kawasaki disease
  • Takayasu's arteritis
  • Wegener's granulomatosis
  • rapidly progressive glomerulonephritis

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

Blood tests

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Blood tests are necessary to accurately diagnose vasculitis.

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 take to 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.
  • Platelet count: Platelets are cells in the blood that clump together to stop bleeding. Platelets form part of our blood's clotting or coagulation system.
  • A complete blood cell count - In addition to n anti-neutrophil cytoplasmic antibodies test.

Urine tests

These may reveal the amount of protein in the urine, or if there are any red blood cells. Urine tests can determine whether there is a kidney problem.

Imaging scans

These may be used to have a look at the larger arteries and their branches. X-rays, ultrasounds, CT scans, or MRI scans may be used.

Angiogram

An angiogram is an X-ray of the blood vessels. A long thin, flexible tube, or catheter, is inserted into a large vein or artery. A dye is injected into the blood vessels through the catheter. The dye shows up in X-ray images allowing the doctor to see the blood vessels.

Biopsy

A biopsy is when a doctor surgically removes a small sample of the affected blood vessel. This is then examined for signs of vasculitis.