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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 known as secondary vasculitis.

Arthritis Curehow to Arthritis Cure for Read more about vasculitis in the Vasculitis Routemap, produced by Vasculitis UK.

Arthritis Curehow to Arthritis Cure for 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:

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

Causes

There''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 the 1 last update 2020/05/28 trigger (such as an infection or drugs) to start this off.There''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 defence mechanisms aren''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''ve been taking and your general health during the past few weeks.

What tests are there?

Arthritis Curehow to Arthritis Cure for There are many tests that may be done to help diagnose the condition. In this the 1 last update 2020/05/28 section we''re not sure about what a certain test will involve.There are many tests that may be done to help diagnose the condition. In this section we''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 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 a dangerous loss of blood supply to the arms or legs or any major organs.

Arthritis Curehow to Arthritis Cure for 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.

Giant cell arteritis (temporal arteritis)

Giant cell arteritis, or GCA, affects the large arteries that supply the head and neck, especially the temporal artery which is found over the temples. There are around 5,000 new cases a year in the UK and it''t normally affect people below the age of 50.

GCA can cause headaches and is often associated with a condition called polymyalgia rheumatica (PMR), which causes inflammation and stiffness in the muscles for 1 last update 2020/05/28 of the shoulders and hips.GCA can cause headaches and is often associated with a condition called polymyalgia rheumatica (PMR), which causes inflammation and stiffness in the muscles of the shoulders and hips.

GCA occasionally involves the blood supply to the eye, where it can cause blindness. If you develop symptoms in your eyes, such as blurring or double vision, you should see your doctor straight away as you''s sometimes called mucocutaneous lymph node syndrome (because it involves the mucous membrane).

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

This condition is quite rare but can be serious if the arteries supplying the heart are inflamed (coronary arteritis). Up to 60% of people with Kawasaki disease have coronary arteritis.

Granulomatosis with polyangiitis

The condition granulomatosis with polyangiitis is quite rare – altogether there are only 1,000 new cases of granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis a for 1 last update 2020/05/28 year in the UK. It''re not recognised early.The condition granulomatosis with polyangiitis is quite rare – altogether there are only 1,000 new cases of granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis a year in the UK. It''re not recognised early.

Arthritis Curehow to Arthritis Cure for The name of this disease has changed recently. It was previously called Wegener''s granulomatosis, granulomatiosis with polyangiitis, and GPA describe exactly the same disease.

Behçet's syndrome

Behçet''s disease (pronounced betchets) is a rare autoimmune condition that can involve mouth ulcers, genital ulcers, skin problems and eye inflammation. It''t be passed from one person to another.

It can also involve other areas of the body, such as the gastrointestinal tract, as well as the pulmonary, musculoskeletal, cardiovascular and neurological systems.

Arthritis Curehow to Arthritis Cure for Because of the many different parts of the body that can be the 1 last update 2020/05/28 affected with Behçet''s also a higher risk of your heart being involved, which can sometimes cause damage to the heart muscle similar to the damage that occurs during a heart attack.Because of the many different parts of the body that can be affected with Behçet''s also a higher risk of your heart being involved, which can sometimes cause damage to the heart muscle similar to the damage that occurs during a heart attack.

This disease was previously called Churg-Strauss syndrome after the doctors who were thought to have discovered it. The new name describes the features of the disease – eosinophils, granulomata and involvement of many blood vessels.

Microscopic polyangiitis

Almost all people with microscopic polyangiitis have kidney problems that can lead to raised blood pressure and kidney failure.

People may find that they''t need 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.

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''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''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 instructions carefully. 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''diet''t usually need to keep to any special diet.

A healthy, low-fat, nutritious and balanced diet is important for everyone, but if you''s particularly important because these can increase your appetite and cause weight gain. Try not to over eat, and cut down on fatty and sugary foods and others which are particularly high in calories. Instead, eat lots of fresh fruit and vegetables and starchy foods like potatoes and wholemeal bread, pasta and rice.

Drinking plenty of water is helpful. It''re taking steroids then you''stop-smoking''s therefore very important to try to stop smoking. This will also improve any symptoms of Raynaud''keeping-warm''s phenomenon. Wearing warm clothes, including warm socks and gloves, should improve blood circulation to your hands and feet by helping to keep the blood vessels open.

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