Vasculitis

Vasculitis means inflammation of the blood vessels. It is an inflammatory condition that can affect any of the body's blood vessels, causing a variety of symptoms and potential complications. Inflammation causes swelling of the blood vessel walls, reducing or even blocking the flow of blood to the body's tissues and organs.

The damage caused by vasculitis depends on which part of the body is affected. The larger the blood vessels that are affected, the greater the potential damage. And the more important the body tissue that is affected, 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 internal bleeding. Apart from the damage to the blood vessel itself, this can lead to damage in the tissues or organs supplied by the blood vessel.

 

Vasculitis can occur on its own (primary vasculitis) or in association with other conditions (including rheumatoid arthritis, systemic lupus erythematosus (SLE) or Sjögren's syndrome) in which case it is known as secondary vasculitis.

Read entire article »

Vasculitis symptoms

When any part of your body is inflamed, it swells and is uncomfortable or painful. With many types of vasculitis, the swelling is internal and there are no external symptoms visible.

Vasculitis takes different forms and the symptoms vary from person to person. Many people with vasculitis feel unwell with fever, sweats, fatigue and weight loss.

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

  • Skin – Vasculitis in the skin causes a rash of spots that sometimes rupture leaving open sores (ulcers)
  • Lungs – Inflammation of the lungs causes coughing and shortness of breath
  • Nerves – Inflammation of the nerves can cause tingling (pins and needles) or weakness in the arms and legs
  • Kidneys – When vasculitis affects the kidneys there may be problems passing urine or there may be blood in the urine. Vasculitis of the kidneys can be dangerous because the symptoms may not appear until the kidneys have already been damaged
  • Fingers and toes – Some people with vasculitis experience Raynaud's phenomenon where the fingers or toes turn white or blue, tingle or hurt when exposed to cold conditions.

What types of vasculitis are there?

There are a number of different types of vasculitis. These include:

Takayasu's arteritis

This affects the main artery from the heart and its large branches, usually in younger women. It's rare in the UK but is more common in the Far East and Africa.

The major arteries narrow and this reduces the blood supply to the limbs and other parts of the body. 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.

Temporal arteritis (or giant cell arteritis)

This affects the large arteries that supply the head and neck, especially the temporal artery (over the temples). It's more common in northern Europe, and in people over the age of 50.

Temporal arteritis frequently causes headaches, and is quite often associated with a condition called polymyalgia rheumatica which causes inflammation and stiffness in the muscles of the shoulders and hips. Temporal arteritis occasionally involves the blood supply to the eye where it can cause blindness. Symptoms in your eyes, such as blurring or double vision, should be reported to your doctor straight away as rapid treatment is needed.

Polyarteritis nodosa ( PAN )

This condition is potentially very serious but fortunately very rare – only about one out of every two million people in the UK develop PAN each year. It causes inflammation in the medium-sized arteries, particularly those supplying the gut and kidneys. This may only affect part of the wall of the artery, which becomes weak and may bulge, forming an aneurysm. If it bursts it can cause serious internal bleeding. Alternatively, it can involve the whole wall of the artery at a particular point, which causes a blockage.

Kawasaki disease

Kawasaki disease affects small and medium-sized arteries in young children (aged under 5 years). It's sometimes called mucocutaneous lymph node syndrome (because it involves the mucous membrane).

Children with Kawasaki disease often feel generally unwell – they may have a high temperature, swollen glands in the neck (lymphadenopathy), an inflamed area around the eye (conjunctivitis) and the mouth, and a skin rash which is similar to measles.

Although relatively rare, this condition can be serious if the arteries supplying the heart are inflamed (coronary arteritis). Up to 60 per cent of patients with Kawasaki disease have coronary arteritis.

Wegener's granulomatosis

This is relatively rare and is slightly more common in men than in women. It usually develops with ear, nose and throat problems, including nosebleeds and crusting of the nose, and occasionally coughing up blood (haemoptysis).

These symptoms can appear a year or two before more general vasculitis starts. This general vasculitis usually involves the skin, lungs, eyes and kidneys. Many people with this disease have kidney problems which, if not recognized early, can sometimes lead to kidney failure.

Churg–Strauss syndrome

In this condition asthma develops in adult life, followed by inflammation of the blood vessels caused by swellings called granulomas. There will also usually be a high number of eosinophils (a particular type of white cell) in the blood. The condition differs from Wegener's granulomatosis because of the allergic history (asthma). Unlike Wegener's granulomatosis, it rarely causes damage to the ears and nose.

Churg–Strauss syndrome usually affects the nerves causing weakness, pins and needles or numbness. There's also a higher risk of the heart being involved which can occasionally cause damage (necrosis) to the heart muscle similar to the damage that occurs during a heart attack.

Microscopic polyangiitis

Almost all people with this condition have kidney problems that could lead to kidney failure. Patients usually find that they are always tired because of anaemia. Blood tests will show that the kidney is inflamed. Microscopic polyangiitis can also involve the lungs, with bleeding that can cause breathlessness.

Cryoglobulin-associated vasculitis

In this disease, small-vessel vasculitis is associated with cryoglobulins – these are proteins in the blood that stick together in the cold. Too many cryoglobulins can reduce the flow of blood or even block the blood vessels causing damage to the organs or body tissues.

Henoch–Schönlein purpura

Henoch–Schönlein purpura (HSP) affects the small blood vessels (capillaries). It often follows an acute chest infection and may be an allergic reaction to a virus, or to food or drugs.

It mostly affects children aged 2–10 years, and boys are affected more often than girls. However, adults can also be affected.

The symptoms of HSP include:

  • a skin rash – spots are red initially but develop into a bruised purple colour and appear in crops over a period of several days or even weeks
  • arthritis, especially of the larger joints, which usually resolves itself within a few days
  • abdominal pain and/or vomiting or passing blood in stools
  • passing blood in urine – indicating kidney problems
  • fever, headaches and loss of appetite.

In most cases the condition resolves itself without specific treatment, although relapses are possible for up to a year after the original illness. Kidney problems are quite common, but serious kidney damage is rare. Occasionally other blood vessels are involved, and rarely more serious complications can occur, sometimes affecting the bowels or causing seizures.

Who gets vasculitis?

About 3,000 people in the UK develop vasculitis each year. The different types of vasculitis tend to affect different age groups.

  • Temporal arteritis, which affects the arteries of the head, is much more common in people over 50
  • Takayasu's arteritis, which affects the main artery from the heart, tends to affect younger women
  • Henoch–Schönlein purpura (HSP), which is an allergic form of vasculitis mainly affecting the skin, is much more common in children than in adults.

What causes vasculitis?

There is no single cause of vasculitis, and in most cases the exact cause is unknown.

We know that vasculitis isn't directly inherited – but genetic factors do play a part as several cases can occur in the same family.

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.

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

How is vasculitis diagnosed?

As infections, drugs and foods can sometimes cause vasculitis, your doctor will probably ask about anything unusual you have taken or eaten during the past few weeks.

What tests are there?

Blood tests may be used to measure inflammation including erythrocyte sedimentation rate (ESR) and 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 Wegener's granulomatosis, while tests such as the CKMB enzyme level can show whether the heart is involved.

Immunological blood tests can be very helpful when the diagnosis is uncertain.

If you have vasculitis along with other diseases, such as rheumatoid arthritis and systemic lupus erythematosus (SLE), 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 systemic lupus erythematosus.

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 – These will show the presence of blood and/or protein, which are often the first signs of an inflamed kidney
  • A urea and electrolytes (U&E) test or a creatinine test may be used to check how your kidneys are working
  • People with Churg–Strauss syndrome, Wegener's granulomatosis or microscopic polyangiitis will have regular urine tests for blood and protein.
  • Liver function tests may also be carried out
  • X-rays can be used to check for chest problems
  • Echocardiogram and electrocardiogram – The heart can be assessed by a special ultrasound test known as an echocardiogram and an electrical test, the electrocardiogram, or ECG)
  • A biopsy may be needed to confirm whether the kidneys, the muscles or lungs are affected by vasculitis. A small piece of tissue is removed from the organ in question for examination or testing
  • An ear, nose and throat (ENT) assessment may be needed for people with Wegener's granulomatosis 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.

Vasculitis treatments

The treatments used for vasculitis will depend upon which blood vessels and which organs are affected. If the vasculitis only affects the skin, it may be sufficient to treat any underlying infection or to remove the drug or food that triggered the vasculitis.

However, in most cases, drug treatments will be needed to control the disease and its symptoms, and to limit the damage caused by vasculitis.

Vasculitis drugs

Corticosteroid drugs (steroids), which can be taken as tablets or given by injection, are effective in many forms of vasculitis, and can be combined with other treatments if necessary.

If you have small-vessel vasculitis that only affects the skin, then you may only need a small dose of steroids to control it, if treatment is needed at all.

Steroids are also effective against vasculitis that affects the larger blood vessels, and are usually given by themselves, for example, for Takayasu's arteritis and temporal arteritis. They can also reduce the risk of temporal arteritis in people who have polymyalgia rheumatica, and this risk may be further reduced by the addition of aspirin.

If you have vasculitis that could affect the kidneys, for example Wegener's granulomatosis, then you are likely to need immunosuppressive drugs in addition to steroids. Treatment will probably be in stages (although steroids may be used throughout):

  • An immunosuppressive drug, usually cyclophosphamide, is used to bring the disease under control by suppressing the immune system that is attacking the blood vessels
  • A milder drug, usually azathioprine, may then be used to keep the disease under control. Methotrexate or mycophenolate are sometimes used instead of azathioprine.

A small minority of people with the most severe types of vasculitis – where there is severe kidney disease, for example – may need a treatment known as plasma exchange or 'blood washing'. This means you are connected to a machine and your blood is passed through the machine and returned to your body after being cleaned of the factors causing the vasculitis – for example, cryoglobulins in cryoglobulin-associated vasculitis. In cases of severe kidney damage, then treatment on an artificial kidney (dialysis) machine may sometimes be necessary.

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

  • Kawasaki disease can be treated effectively with injections of globulin (a type of protein)
  • Hepatitis-associated polyarteritis nodosa can be treated with anti-viral treatment and plasma exchange.

If you need high doses of steroids as part of your treatment, then your doctor may suggest additional drugs, such as bisphosphonates to guard against osteoporosis, which can sometimes occur as a side-effect of steroid treatments.

In some types of vasculitis, such as Wegener's granulomatosis, anti-bacterial drugs such as co-trimoxazole may be given to protect against infections that could trigger a relapse. These drugs can also help to protect against possible side-effects of the stronger immunosuppressive drugs.

Studies with biologic drugs such as rituximab aim to establish whether these drugs could be helpful for people with certain types of vasculitis, including Wegener's granulomatosis and Takayasu's arteritis.

Self-help and daily living

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. It's also important to make sure the treatment is not interrupted.

This section covers: exercise, diet and nutrition, smoking and keeping warm.

Exercise

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) but swimming is also recommended.

Diet and nutrition

You will not usually need to keep to any particular diet, unless the vasculitis has been triggered by a food.

A healthy, balanced diet is important for everyone, but if you are on steroids it is particularly important because these can increase your appetite and cause weight gain. Steroids can also make osteoporosis (thin bones) more likely, so it is important to get enough calcium in your diet. Foods which are good sources of calcium include tinned sardines (with bones), skimmed milk, yoghurt and certain vegetables such as broccoli.

Stop smoking

Avoid smoking. It makes the blood vessels constrict (become narrower inside) and can therefore make vasculitis symptoms worse.

Keep warm

If your fingers or toes turn blue in response to the cold, this may be due to Raynaud's phenomenon. Wearing warm clothes, including warm socks and gloves, should improve blood circulation to the hands and feet by helping to keep the blood vessels open.

Share |