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.

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).

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.

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.

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.

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.

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.

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's more common in northern Europe. GCA doesn'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 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'll need to be treated urgently.

Other blood vessels, such as the major arteries, can less commonly be involved in GCA.

Polyarteritis nodosa

Polyarteritis nodosa (PAN) can be very serious, but it is very rare – only about one out of every two million people in the UK develop PAN each year. In some cases PAN can be associated with hepatitis B virus infection.

PAN causes inflammation in the medium-sized arteries, particularly those supplying blood to the gut and kidneys. This may only affect part of the wall of the artery, which becomes weak and may bulge, forming an aneurism. If it bursts it can cause serious internal bleeding.

PAN can also 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, most commonly in children under five. It'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 year in the UK. It's 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. The kidney problems can sometimes lead to kidney failure if they're not recognised early.

The name of this disease has changed recently. It was previously called Wegener's granulomatosis after the doctor who was thought to have discovered it. The new name describes the features of the disease:

• polyangiitis means that many (poly = many) arteries are involved
• granulomatosis means that when a biopsy of the inflamed tissue is studied under a microscope you can see swellings called granulomata.

Remember that the terms Wegener's granulomatosis, granulomatiosis with polyangiitis, and GPA describe exactly the same disease.

Behçet's syndrome

Behçet's syndrome, or Behçet's disease (pronounced betchets) is a rare autoimmune condition that can involve mouth ulcers, genital ulcers, skin problems and eye inflammation. It's not an infection and can'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.

Because of the many different parts of the body that can be affected with Behçet's, many different medications can be used to control symptoms.

Eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis causes asthma to develop in adults, 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 granulomatosis with polyangiitis because of the asthma. Unlike granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis rarely causes damage to your ears and nose.

Eosinophilic granulomatosis with polyangiitis can also affect the nerves, causing weakness, pins and needles or numbness.

There'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're tired because of anaemia. Anaemia is a shortage of haemoglobin (oxygen-carrying pigment) in the blood which makes it more difficult for the blood to carry oxygen around the body.

Blood tests can show whether the kidney is inflamed. Microscopic polyangiitis can also involve the lungs, with bleeding that can cause breathlessness.

Cryoglobulin-associated vasculitis

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

Immunoglobulin A vasculitis

Immunoglobulin A vasculitis, also known as IgA vasculitis, affects the small blood vessels (capillaries). It often follows a chest infection and may be an allergic reaction to a virus, food or drugs. It mostly affects children aged 2–10 years, and boys are affected more often than girls.

It can also affect adults, but most of the 2,500 new cases a year in the UK are children.

The symptoms of IgA vasculitis include:

• a skin rash, often over the buttocks (starts red but develops into a bruised purple colour and appears over several days or even weeks)
• short-lived arthritis, especially of the larger joints
• stomach pain and/or vomiting or passing blood in stools
• passing blood in urine (indicating kidney problems)
• fever, headaches and loss of appetite.

The rash seen in IgA vasculitis is called purpura and was described by doctors Henoch and Schonlein, which explains the previous name of the disease.

In most cases the condition doesn'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.

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.

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.

Diet

You won't usually need to keep to any special diet.

A healthy, low-fat, nutritious and balanced diet is important for everyone, but if you're on steroids it'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's healthy for everyone not to drink more than the recommended units of alcohol a week; 14 a week for both men and women. Having at least two alcohol-free days is advisable, but not if that means you then save up all your units to drink in one go.

If you're taking steroids then you're more at risk of osteoporosis, but having plenty of calcium in your diet is important to help prevent this from developing. Foods that are good sources of calcium include tinned sardines (with bones), skimmed milk, yoghurt and certain vegetables such as broccoli.

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.

Keeping 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 your hands and feet by helping to keep the blood vessels open.