What is vasculitis?

Vasculitis means inflammation of the blood vessels, the tubes that carry blood around your body. There are three types of blood vessel which can be affected by vasculitis:

  • arteries – take blood from the heart to different parts of the body such as organs (for example kidneys) and tissues (for example skin)
  • veins – take blood back to the heart
  • capillaries – tiny vessels between the arteries and the veins where oxygen and other materials pass from the blood into the tissues.

The organs and tissues in your body need a regular blood supply to work properly. Inflammation causes swelling of the blood vessel walls, reducing or even blocking the flow of blood to the tissues and organs.

Vasculitis can cause a range of symptoms and possible complications. The amount of damage vasculitis causes depends on which part of the body is affected. The larger the affected blood vessels, the more damage there may be; the more important the affected body tissue, the more serious the damage will be.

The walls of affected blood vessels can swell and bulge (this is called an aneurysm) and may even burst, causing bleeding inside your body. Apart from the damage to the blood vessel itself, this can lead to damage in the tissues or organs which are supplied by the blood vessel.

Vasculitis can occur suddenly in someone who has previously been completely well – when it occurs on its own, doctors call this primary vasculitis. Vasculitis can also occur alongside other conditions (including rheumatoid arthritis, lupus or Sjögren's syndrome), in which case it's known as secondary vasculitis.

Read more about vasculitis in the Vasculitis Routemap, produced by Vasculitis UK.

Who gets it?

Vasculitis is uncommon. Overall, in every 100,000 people in the UK, only 10–15 will develop vasculitis each year. However, about 22 people per 100,000 aged over 50 years will develop giant cell arteritis (GCA)*. The different types of vasculitis tend to affect different age groups, for example:

  • GCA (temporal arteritis) is much more common in people over 50, and it's fairly common for it to be associated with a condition called polymyalgia rheumatica (PMR).
  • Takayasu arteritis (TA) tends to affect younger Asian women.
  • IgA vasculitis is much more common in children than in adults.
  • Kawasaki disease only affects children under five.

*Smeeth L, Cook C, Hall AJ. Incidence of diagnosed polymyalgia rheumatica and temporal arteritis in the United Kingdom, 1990–2001. Ann Rheum Dis 2006;65:1093–8.

Symptoms

When any part of your body is inflamed, it swells and is uncomfortable or painful. With many types of vasculitis the swelling is inside the body and you can't see any of the symptoms on the outside.

Vasculitis takes different forms according to which blood vessels are affected, and symptoms vary. Many people with vasculitis feel unwell and have fever, sweats, fatigue and weight loss. These can be the first symptoms experienced, so it's important to be seen by your GP.

Other symptoms vary according to which part of the body is affected, for example:

  • Skin – vasculitis in the skin causes spots that can burst, leaving open sores (ulcers). When vasculitis only affects the skin, long-term effects aren't usually serious, and symptoms normally clear up once inflammation has settled.
  • Nose – vasculitis here causes crusting inside the nose and nosebleeds. Sometimes the shape of the nose can change.
  • Fingers and toes – some people with vasculitis experience Raynaud's phenomenon, where the fingers or toes turn white or blue and may tingle or hurt when exposed to cold conditions.
  • Eyes – some types of vasculitis can suddenly affect your vision or cause your eye/s to become red or painful. It's very important to see a doctor quickly if this happens.
  • Nerves – inflammation of the nerves can cause tingling (pins and needles), pain and burning sensations or weakness in the arms and legs.
  • Joints – vasculitis can cause joint pain or swelling.
  • Muscles – inflammation here causes muscle aches, and eventually your muscles could become weak.
  • Lungs – inflammation of the lungs causes coughing and shortness of breath.
  • Brain – occasionally the blood vessels in the brain can be affected, causing problems like strokes.
  • Kidneys – when vasculitis affects the kidneys there may be problems passing urine or blood in the urine. Vasculitis of the kidneys can be dangerous as symptoms may not appear until the kidneys have been damaged. In severe cases treatment on an artificial kidney (dialysis) machine may be necessary.

Headaches, pain in the jaw and problems with the eyes can be serious symptoms of giant cell arteritis (GCA).

Causes

There's no single cause of vasculitis, and in most cases the exact cause is unknown. We know that vasculitis isn't directly inherited through the genes we get from our parents, but genetic factors do play a part as several cases can occur in the same family. Your genes could make you more likely to develop the condition, in which case it may only take a small trigger (such as an infection or drugs) to start this off.

We also know that some types of vasculitis – for example, those affecting the small blood vessels – can be related to infections, particularly those associated with hepatitis. Some cases of vasculitis occur after treatment with certain drugs, for example propylthiouracil (used to treat thyroid disease) and allopurinol (used to treat gout), as well as non-steroidal anti-inflammatory drugs (NSAIDs) and antibiotics.

It's thought that most forms of vasculitis are a type of autoimmune disease. This means that your body's defence mechanisms aren't doing their normal job of fighting infections, but instead attack a healthy part of the body, causing inflammation.

How will vasculitis affect me?

The most severe types of vasculitis can be life-threatening. Early diagnosis and treatment is essential for the best chances of avoiding permanent damage to tissues and organs.

Most types of vasculitis respond well to treatment, and for many of them you're likely to make a full recovery, although relapses in the future are possible.

The outcome depends on the type of vasculitis and how it affects you. Overall, the best way to learn more about what might happen to you in the future is to talk to your doctor or another health professional.

Who diagnoses and treats vasculitis?

If you have vasculitis it's important you have one consultant who is in overall charge of your care. The management and care of patients with vasculitis is usually undertaken mainly by a rheumatologist, as they specialise in inflammatory diseases, or less frequently by a nephrologist (kidney doctor), especially if the kidneys are affected.

However, vasculitis in some circumstances may affect a variety of different organs. If required, your care should involve a multi-disciplinary team of consultants with different specialities, working together. For example, if your lungs and airways are seriously affected, care may involve a respiratory consultant. In some cases an ear, nose and throat (ENT) consultant, an ophthalmologist, neurologist, gastro-enterologist, immunologist or dermatologist may also be involved.

Diagnosis

If you think you may be developing vasculitis you must see your doctor as soon as possible. Infections, drugs and some foods can sometimes cause vasculitis, so your doctor will probably ask about the medications you've been taking and your general health during the past few weeks.

What tests are there?

There are many tests that may be done to help diagnose the condition. In this section we'll look at the most common, but there are others that you may need. Ask your rheumatologist, other specialist or GP if you're not sure about what a certain test will involve.

Blood tests may be used to measure inflammation – for example, the erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP).

A full blood count can help to establish whether you have anaemia and whether you have normal levels of white blood cells (which fight infections) and platelets (which are involved in clotting).

Blood tests for antineutrophil cytoplasmic antibodies (ANCA) are important in the diagnosis of some types of vasculitis, particularly:

  • granulomatosis with polyangiitis
  • eosinophilic granulomatosis with polyangiitis
  • microscopic polyangiitis.

The following tests may be used to check how your kidneys are working:

  • a urea and elecrolytes (U&E) test
  • an estimated glomerular filtration rate test (eGFR)
  • a creatinine test.

Liver function tests may also be carried out to check how your liver is working.

If you have vasculitis along with other conditions, such as rheumatoid arthritis or lupus, then blood tests might be used to assess how active these other diseases are. Blood tests can measure the level of rheumatoid factor in rheumatoid arthritis, or the levels of complement (an enzyme system or group of proteins in the blood) and antibodies in lupus.

Blood tests may be repeated from time to time to check how your condition is responding to treatment.

Other tests may be carried out to see how the affected body organs are working – for example:

  • Urine tests will show the presence of blood and/or protein, which are often the first signs of an inflamed kidney. People with eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis or microscopic polyangiitis will have regular urine tests for blood and protein.
  • X-rays, CT and MRI scans can be used to check for chest problems.
  • Echocardiograms and electrocardiograms can be used to assess the heart. An echocardiogram is a special ultrasound test and an electrocardiogram (ECG) is an electrical test.
  • A biopsy may be needed to confirm whether the kidneys, muscles, skin or lungs are affected by vasculitis. A small piece of tissue is removed from the organ in question for examination or testing in a laboratory.
  • An ear, nose and throat (ENT) assessment may be needed for people with granulomatosis with polyangiitis who have symptoms in these parts of the body.
  • An angiogram is often done where abdominal organs such as the kidney and gut are involved. This involves injecting dye into the arteries so that they show up on an x-ray. They can also be done in Takayasu arteritis and giant cell arteritis to see how much the large blood vessels are involved.

What types of vasculitis are there?

Doctors usually define the types of vasculitis according to the size of the blood vessels involved. The most serious types of vasculitis involve both small and medium-sized arteries.

Takayasu arteritis

Takayasu arteritis (TA) is an inflammatory disease that affects the main artery from the heart (the aorta) and its large branches, usually in younger women. It's rare in the UK (there are only 100 new cases a year) but it's more common in the Far East and Africa.

TA causes the arteries to narrow, and this can reduce the blood supply. The narrowing develops slowly and the arteries don't usually block completely, so there isn't usually a dangerous loss of blood supply to the arms or legs or any major organs.

Other major arteries can also be affected, including:

  • the carotid arteries in the head and brain
  • coronary arteries in the heart
  • renal arteries to the kidneys
  • arteries that take blood to the arm.

Treatment

The treatments used for vasculitis will depend upon which blood vessels and organs are affected, as well as how much body tissue is affected. If the vasculitis only affects the skin, it may be enough to treat any underlying infection or to remove the drug that triggered the vasculitis. However, in most cases, drug treatment will be needed to control the disease and its symptoms and to stop or limit the damage caused by vasculitis.

Drugs

The two main types of drug used to treat vasculitis are steroids and immunosuppressant agents. Both act to dampen down the immune system to reduce the strength of its attack on the tissues of the body.

If you have vasculitis affecting the large blood vessels, then you'll probably be given steroid tablets. These are very effective for giant cell arteritis and Takayasu arteritis, and often immunosuppressive agents aren't needed for these conditions.

If you have vasculitis affecting small and/or medium-sized blood vessels, then you may only need a small dose of steroids to control it.

However, you may need a combination of steroids and immunosuppressive drugs, probably over several years, especially if vasculitis affects internal organs. For many types of vasculitis, including those affecting the kidney, lungs or other vital organs (especially if it involves both small and medium-sized blood vessels), your treatment will be given in stages.

If you have vasculitis that mainly affects medium-sized arteries, then other treatments can help, depending on the condition:

  • Kawasaki disease can be treated effectively with the injections of immunoglobulin (a type of protein).
  • Hepatitis-associated PAN can be treated with antiviral treatment and plasma exchange.

Plasma exchange (also known as blood washing) involves being connected to a machine that your blood passes through before being returned to you so it can be cleaned of the factors causing the vasculitis. Only a few people with the most severe types of vasculitis – for example who have very severe kidney or lung disease – will need plasma exchange. This will be done in specialist centres.

Possible side-effects of steroids include weight gain, indigestion, diabetes, thinning of the skin and thinning of the bones (osteoporosis). If high doses of steroids are given, then you'll also be given drugs like bisphosphonates to help prevent osteoporosis.

The stages of treatment for some types of vasculitis:

Stage 1: Bringing you to remission. The aim is to get the disease under control. An immunosuppressive drug (for example cyclophosphamide) may be used to dampen down the immune system, which is attacking the blood vessels. Steroid tablets or injections will also be used in this stage.

Stage 2 Keeping you in remissions. Once the condition is in remission, the aim is to keep it under control. Drugs (for example azathioprine, methotrexate or mycophenolate) may be used with steroids.

Stage 3: Following up and withdrawing therapy. The aim at this stage is to gradually reduce your medications while still keeping the disease under control.

In some types of vasculitis (such as granulomatosis with polyangiitis), an infection may trigger a relapse. You may therefore be given antibacterial drugs such as co-trimoxazole to protect against this. These drugs can also help to protect against the increased risk of infection caused by the stronger immunosuppressive drugs.

As with all medications, there may sometimes be side-effects.

Cyclophosphamide, for example, can cause bleeding from the bladder, hair thinning and an increased risk of infection. Unfortunately, there's also a significant risk that it can reduce fertility in both men and women.

Because of these risks, cyclophosphamide will be stopped or exchanged for a different immunosuppressive drug as soon as your vasculitis is controlled. This is usually azathioprine, but methotrexate or mycopheonlate might be used instead. Rituximab, a biological therapy given by intravenous infusion, can also help to encourage remission in some types of vasculitis.

Managing symptoms

Learn how to manage the symptoms of vasculitis.

Exercise

If you do need treatment then it's very important that you follow your doctor's instructions carefully. It's important to learn and understand as much as you can about your illness and the treatment options; you can discuss alternative treatment options with your medical team.

Vasculitis varies from one person to the next and from one type of vasculitis to another. It's important to speak to your doctor or other healthcare professional about any new symptoms you may have.

Vasculitis can cause tiredness, and it's important to rest when you need to. However, you should also try to keep muscles and joints healthy by exercising. Start gently and gradually increase the amount of exercise you do. Include some weight-bearing exercise (anything that involves walking or running). Swimming is also recommended. Ask your doctor for advice on how much exercise you should expect to be able to do.

Living with vasculitis

Stop smoking

Smoking makes the blood vessels become narrower inside and can therefore make vasculitis symptoms worse. Smoking can also worsen lung inflammation. It's therefore very important to try to stop smoking. This will also improve any symptoms of Raynaud's phenomenon. If you want to stop smoking see your doctor who will be able to give you advice about quitting.