sign in sign up
<<
Home , Arteritis, Giant cell arteritis, Vasculitis

Condition

Vasculitis This booklet provides information and answers to your questions about this condition.

Arthritis Research UK booklets are produced and printed entirely from charitable donations. What is vasculitis?

Vasculitis means of the blood vessels. There are several different types of the condition, many with unknown causes, but treatments can be very effective. In this booklet we’ll briefly explain the main types of vasculitis, how they’re diagnosed and treated, what you can do to help yourself and where to get more information.

At the back of this booklet you’ll find a brief glossary of medical words – we’ve underlined these when they’re first used. www.arthritisresearchuk.org Page 2 of 32 Vasculitis information booklet Research UK

What’s inside? 2 Vasculitis at a glance 5 Who diagnoses and treats 15 What is the outlook? vasculitis? 15 How is vasculitis diagnosed? 6 What is vasculitis? – What tests are there? 8 What are the symptoms 17 What treatments are there for vasculitis? of vasculitis? – Drugs 9 What types of vasculitis 20 Self-help and daily living are there? – Exercise – Takayasu (TA) – Diet and nutrition –  arteritis (temporal arteritis) – Stop smoking –  (PAN) – Keep warm –  22 Research and new – Granulomatosis with polyangiitis developments (Wegener’s granulomatosis) 23 Glossary – Behçet’s syndrome – Eosinophilic granulomatosis 26 Where can I find out more? with polyangiitis (Churg–Strauss 28 We’re here to help syndrome) –  – Cryoglobulin-associated vasculitis – Immunoglobulin A vasculitis (Henoch–Schönlein ) 14 Who gets vasculitis? 15 What causes vasculitis?

Page 3 of 32 Vasculitis information booklet At a glance Vasculitis There are several different types of vasculitis but all are treatable. What is vasculitis? Vasculitis means inflammation of the blood vessels. It can affect any of the body’s blood vessels, causing a variety of different symptoms and problems.

• fingers and toes – can turn What are the symptoms? white or blue, tingle or hurt The main symptom of vasculitis is when exposed to cold conditions inflammation, and this can be painful. (Raynaud’s phenomenon). With many forms of the condition the , jaw pain and eye problems inflammation is internal and you can’t (such as double vision or blurring) are all see it. Because vasculitis takes different possible symptoms of a type of vasculitis forms, the symptoms vary from person called (GCA). to person. Many people with vasculitis feel unwell with , sweats, fatigue and weight loss. Sometimes these can What types of vasculitis be the first symptoms you feel, so it’s are there? important to be seen by your GP who Vasculitis can be primary, where it occurs will consider whether you need to be on its own, or secondary, where it occurs referred to a specialist. with another condition. The following are Other symptoms vary according to which types of primary vasculitis: part of the body is affected, for example: • Takayasu arteritis (TA) – a disease that •  – of spots that can rupture, causes inflammation within the large leaving open sores (ulcers) . TA particularly affects the and its main branches. The aorta is the • – coughing or breathlessness main carrying blood from the •  – tingling (pins and needles), heart to the rest of the body. pain and/or weakness in the arms • Giant cell arteritis (temporal arteritis) and legs – affects the large arteries that supply • kidneys – problems passing urine, the head and neck, especially the or blood in the urine temporal artery (very rare in people under 50). Page 4 of 32 Vasculitis information booklet

• Polyarteritis nodosa (PAN) – • Microscopic polyangiitis – causes inflammation in the medium-sized problems that without arteries, such as those supplying the gut treatment can lead to kidney failure, and kidneys. and can also involve the lungs, with • Kawasaki disease – affects small that can cause breathlessness. and medium-sized arteries in young • Cryoglobulin-associated vasculitis children (usually aged under five years). – causes a reduction in blood flow • Granulomatosis with polyangiitis or even a blockage, causing damage (Wegener’s) – involves inflammation to the organs or body tissues. of small and medium-sized arteries • Immunoglobulin A vasculitis in the skin, lungs, eyes and kidneys. (Henoch–Schönlein) – affects small If a is taken, under a microscope, blood vessels () and mostly doctors can see swellings called affects children between 2–10 years; granulomata in this type of vasculitis. symptoms include a skin rash, arthritis, • Behçet’s syndrome, or Behçet’s , and/or vomiting, disease – is a rare autoimmune passing blood in the stools and/or urine, condition that often involves mouth fever, headaches and loss of appetite. ulcers, genital ulcers, skin problems and eye inflammation. Different parts of How is it diagnosed? the body can be involved, so it can be treated with different medications. Vasculitis is diagnosed using blood tests, x-rays or other scans and Eosinophilic granulomatosis with • (getting a tissue sample to look at under polyangiitis (Churg–Strauss) – a microscope). developing in adult life, followed by inflammation of the blood What tests are there? vessels; this form of vasculitis usually The following tests may be used to check affects the nerves, causing weakness, for types of vasculitis: pins and needles and numbness. If a biopsy is undertaken, under a • blood tests – to test for inflammation, microscope, doctors can see swellings low blood count, kidney and liver called granulomatoma. function, or to test for antineutrophil cytoplasmic (ANCA)

Page 5 of 32 Vasculitis information booklet

Only 10–15 out of every 100,000 people will What treatments are there? develop vasculitis Treatments for the various types in the UK of vasculitis include: • drug treatments, including steroid each year. tablets or injections, immunosuppressive drugs, biological therapies, immunoglobulin infusions, antiviral treatments and antibacterial drugs • plasma exchange (sometimes called blood washing), which may be needed by a small number of people with the most severe forms of vasculitis • urine tests – to test for kidney • dialysis in cases of severe kidney damage. inflammation • x-rays, computerised tomography What else might help? (CT) and magnetic resonance imaging (MRI) scans to test for chest There are some things you can do to help or sinus problems yourself if you have vasculitis, including: • heart scans such as echocardiograms • not smoking and electrocardiograms (ECGs) to check • balancing regular exercise with rest your heart is healthy • eating a healthy balanced diet, • a biopsy may be advised to check if including enough calcium and your temporal arteries, liver, kidneys, vitamin D if you’re on steroids muscles or lungs are affected • keeping warm (especially if the • an ear, nose and throat (ENT) vasculitis has caused Raynaud’s examination to check if these parts phenomenon) of the body are affected • learning about the treatment you’re • a special x-ray or MRI scan called an on and what you need to do to reduce angiogram to see which blood vessels your risk of side-effects, including are involved. vaccinations against seasonal flu and pneumonia.

Page 6 of 32 Vasculitis information booklet

Who diagnoses and treats If required, your care should involve a multi-disciplinary team of consultants vasculitis? with different specialities, working If you have vasculitis it’s important you together. For example, if your lungs have one consultant who is in overall and airways are seriously affected, care charge of your care. The management may involve a respiratory consultant. and care of patients with vasculitis In some cases an ear, nose and throat is usually undertaken mainly by a (ENT) consultant, an ophthalmologist, rheumatologist, as they specialise in neurologist, gastro-enterologist, inflammatory diseases, or less frequently immunologist or dermatologist may also by a nephrologist (kidney doctor), be involved. especially if the kidneys are affected. However vasculitis in some circumstances may affect a variety of different organs.

Page 7 of 32 Vasculitis information booklet

What is vasculitis? The organs and tissues in your body need a regular blood supply to work properly. Vasculitis means inflammation of the Inflammation causes swelling of the blood vessels, the tubes that carry blood walls, reducing or even around your body. There are three types blocking the flow of blood to the tissues of blood vessel which can be affected and organs (see Figures 2 and 3). by vasculitis (see Figure 1): Figure 1: The three types of blood vessel that can be affected by vasculitis

Arteries Capillaries

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

Figure 2 The blood circulatory system. Heart Large artery Kidneys

Small arteries Small veins The capillaries, which are too small to be seen with Medium-sized your eye, are spread artery throughout the tissues in your body.

Page 8 of 32 Vasculitis information booklet

Vasculitis can cause a range of symptoms Vasculitis can appear suddenly in and possible complications. The amount someone who has previously been of damage vasculitis causes depends on completely well – when it occurs on its which part of the body is affected. The own, doctors call this primary vasculitis. larger the affected blood vessels, the Vasculitis can also occur alongside other more damage there may be; the more conditions (including , important the affected body tissue, the or Sjögren’s syndrome) in which more serious the damage will be. case it’s known as secondary vasculitis. The walls of affected blood vessels can swell and bulge (this is called an ) See Arthritis Research UK booklets and may even burst, causing bleeding Lupus (SLE); Rheumatoid arthritis; inside your body. Apart from the damage Sjögren’s syndrome. to the blood vessel itself, this can lead to damage in the tissues or organs that are supplied by the blood vessel.

Figure 3 Vasculitis means inflammation Normal of the blood blood flow vessels.

Normal artery wall

The diagram Reduced shows a normal blood flow artery (top) and one that’s inflamed (bottom). Inflamed and thickened artery wall

Page 9 of 32 Vasculitis information booklet The symptoms of vasculitis vary according to which part of the • Fingers and toes – some people body is affected. 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 What are the symptoms nerves can cause tingling (pins and of vasculitis? needles) pain and burning sensations When any part of your body is inflamed, or weakness in the arms and legs. it swells and is uncomfortable or painful. • Joints – vasculitis can cause joint pain With many types of vasculitis the swelling or swelling. is inside the body and you can’t see any • Muscles – inflammation here causes symptoms on the outside. muscle aches, and eventually your Vasculitis takes different forms according muscles could become weak. to which blood vessels are affected, • Lungs – inflammation of the lungs and symptoms vary. Many people with causes coughing and shortness vasculitis feel unwell and have fever, of breath. sweats, fatigue and weight loss. These can be the first symptoms experienced, so it’s • Brain – occasionally the blood vessels important to be seen by your GP. Other in the brain can be affected, causing symptoms vary according to which part of problems like . the body is affected: • Kidneys – when vasculitis affects the • Skin – vasculitis in the skin causes kidneys there may be problems passing spots that can burst, leaving open sores urine or blood in the urine. Vasculitis (ulcers). When vasculitis only affects the of the kidneys can be dangerous as skin, long-term effects aren’t usually symptoms may not appear until the serious, and symptoms normally clear up kidneys have been damaged. In severe once inflammation has settled. cases treatment on an artificial kidney (dialysis) machine may be necessary. • Nose – vasculitis here causes crusting inside the nose and . Headaches, pain in the jaw and problems The shape of your nose can change. with the eyes can be serious symptoms of giant cell arteritis (GCA).

Page 10 of 32 Vasculitis information booklet

What types of vasculitis TA causes the arteries to narrow, and this can reduce the blood supply. are there? The narrowing develops slowly and the Doctors usually define the types of arteries don’t usually block completely, vasculitis according to the size of the so there isn’t usually a dangerous loss of blood vessels involved (see Figure 4). blood supply to the arms or legs or any The most serious types of vasculitis major organs. Other major arteries can involve both small and medium arteries. also be affected, including the carotid arteries in the head and brain, coronary Takayasu arteritis (TA) arteries in the heart, renal arteries to Takayasu arteritis (TA) is an inflammatory the kidneys and arteries that take blood disease that affects the main artery to the arm. 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 is more common in the Far East and Africa.

Figure 4 Types of vasculitis can be defined according to the size of the blood vessels involved.

Vasculitis in large arteries: Vasculitis in medium-sized arteries: • includes giant cell arteritis (temporal • includes polyarteritis nodosa and arteritis) and Takayasu arteritis. Kawasaki disease.

Vasculitis in small vessels: • includes granulomatosis with • can also be a result of rheumatic polyangiitis, microscopic polyangiitis diseases and eosinophilic granulomatosis • includes immunoglobulin A with polyangiitis vasculitis and cryoglobulin– • can happen with associated vasculitis including hepatitis and, very • usually involves the skin and is also occasionally, with different types sometimes caused by a reaction to of certain drugs.

Page 11 of 32 Vasculitis information booklet

Giant cell arteritis GCA occasionally involves the blood (temporal arteritis) supply to the eye, where it can cause blindness. If you develop symptoms Giant cell arteritis, or GCA, affects the in your eyes, such as blurring or large arteries that supply the head and double vision, you should see your neck, especially the temporal artery which doctor straight away as you’ll need is found over the temples (see Figure to be treated urgently. 5). There are around 5,000 new cases a year in the UK and it’s more common in Other blood vessels, such as the major northern Europe. GCA doesn’t normally arteries, can less commonly be involved affect people below the age of 50. in GCA. GCA can cause headaches and is often associated with a condition called See Arthritis Research UK booklets (PMR), which Giant cell arteritis (temporal arteritis); causes inflammation and stiffness in Polymyalgia rheumatica (PMR). the muscles of the and hips.

Figure 5 Headaches can be caused by giant cell arteritis (temporal arteritis).

Photography used with kind permission of Elsevier. © Elsevier 2011. Taken from Practical , third edition. Edited by Marc C Hochberg, Alan J Silman, Josef S Smolen, Michael E Weinblatt and Michael H Weisman. Page 12 of 32 Vasculitis information booklet The type of vasculitis is usually defined by the size of the blood Polyarteritis nodosa (PAN) vessels involved. PAN can be very serious but 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 . 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 granulomatosis with polyangiitis, and may bulge, forming an aneurysm. eosinophilic granulomatosis with If it bursts it can cause serious internal polyangiitis and microscopic polyangiitis bleeding. PAN can also involve the whole a year in the UK. It’s slightly more wall of the artery at a particular point, common in men than in women. which causes a blockage. It usually develops with ear, nose and Kawasaki disease throat problems, including nosebleeds and crusting of the nose, and occasionally Kawasaki disease affects small and coughing up blood (haemoptysis). medium-sized arteries most commonly in These symptoms can appear a year or children under five. It’s sometimes called mucocutaneous lymph node syndrome two before more general vasculitis starts. (because it involves the mucous This general vasculitis usually involves the membrane). skin, lungs, eyes and kidneys. The kidney problems can sometimes lead to kidney Children with Kawasaki disease will failure if they’re not recognised early. feel unwell – they may have a high temperature, swollen glands in the neck The name of this disease has changed (lymphadenopathy), an inflamed area recently. It was previously called around the eye and the mouth, and a skin Wegener’s granulomatosis after the rash similar to measles. doctor who was thought to have discovered it. Polyangiitis means that This condition is quite rare but can be many (poly = many) arteries are involved serious if the arteries supplying the heart and granulomatosis means that when a are inflamed (coronary arteritis). Up to biopsy of the inflamed tissue is studied 60% of people with Kawasaki disease under a microscope you can see swellings have coronary arteritis. called granulomata. Remember that Granulomatosis with polyangiitis the terms Wegener’s granulomatosis, (Wegener’s granulomatosis) granulomatosis with polyangiitis, and GPA describe exactly the same disease. The condition granulomatosis with polyangiitis is quite rare – altogether there are only 1,000 new cases of Page 13 of 32

Vasculitis information booklet

Behçet’s syndrome muscle similar to the damage that occurs Behçet’s syndrome, or Behçet’s disease during a heart attack. (pronounced betchets) is a rare This disease was previously called autoimmune condition that can involve Churg–Strauss syndrome after the doctors mouth ulcers, genital ulcers, skin who were thought to have discovered it. problems and eye inflammation. It’s The new name describes the features of not an infection and can’t be passed the disease – , granulomata and from one person to another. It can also involvement of many blood vessels. involve other areas of the body such as the , as well Microscopic polyangiitis as the pulmonary, musculoskeletal, Almost all people with microscopic cardiovascular and neurological systems. polyangiitis have kidney problems Because of the many different parts of the that can lead to raised body that can be affected with Behçet’s, and kidney failure. People may find many different medications can be used that they’re tired because of anaemia. to control symptoms. Blood tests will show that the kidney is inflamed. Microscopic polyangiitis can Eosinophilic granulomatosis also involve the lungs, with bleeding that with polyangiitis (Churg–Strauss can cause breathlessness. syndrome) Eosinophilic granulomatosis with Cryoglobulin–associated vasculitis polyangiitis causes asthma to develop in In cryoglobulin–associated vasculitis, adults, followed by inflammation of the small-vessel vasculitis is associated with blood vessels caused by swellings called cryoglobulins – these are proteins in the . There will also usually be blood that stick together in the cold. a high number of eosinophils (a particular Having cryoglobulins can reduce the type of white cell) in the blood. flow of blood or even block the blood The condition differs from granulomatosis vessels, causing damage to the organs with polyangiitis because of the asthma. or body tissues. Unlike granulomatosis with polyangiitis, Immunoglobulin A vasculitis eosinophilic granulomatosis with (Henoch–Schönlein purpura) polyangiitis rarely causes damage to your Immunoglobulin A vasculitis, also known ears and nose. as IgA vasculitis, affects the small blood Eosinophilic granulomatosis with vessels. It often follows a chest infection polyangiitis can also affect the nerves, and may be an allergic reaction to a virus, causing weakness, pins and needles or food or drugs. It mostly affects children numbness. There’s also a higher risk of aged 2–10 years, and boys are affected your heart being involved, which can more often than girls. It can also affect sometimes cause damage to the heart adults, but most of the 2,500 new cases a year in the UK are children. Page 15 of 32 Vasculitis information booklet

The symptoms of IgA vasculitis include: In most cases the condition doesn’t need • a rash, often over the buttocks (starts specific treatment, although relapses red but develops into a bruised purple are possible for up to a year after the colour and appears over several days or original illness. Kidney problems are quite even weeks) common, but serious kidney damage is rare. Occasionally other blood vessels • short-lived arthritis, especially of the are involved, and rarely more serious larger joints complications can occur, sometimes • stomach pain and/or vomiting affecting the bowels or causing seizures. or passing blood in stools • passing blood in urine (indicating kidney problems) Who gets vasculitis? Vasculitis is uncommon. In every 100,000 • fever, headaches and loss of appetite. people in the UK, only 10–15 will develop The rash seen in IgA vasculitis is called vasculitis each year. However, about purpura and was described by Drs 22 people per 100,000 aged over 50 years Henoch and Schönlein, which explains will develop GCA. The different types the previous name of the disease. of vasculitis tend to affect different age groups, for example: Vasculitis information booklet

• GCA (temporal arteritis) is much more This means that your body’s defence common in people over 50, and it’s mechanisms aren’t doing their normal fairly common for it to be associated job of fighting infections, but instead with a condition called polymyalgia attack a healthy part of the body, rheumatica (PMR). causing inflammation. • Takayasu arteritis tends to affect younger Asian women. See Arthritis Research UK booklet and drug leaflet Gout; Allopurinol. • IgA vasculitis is much more common in children than in adults. • Kawasaki disease only affects children under five. What is the outlook? The most severe types of vasculitis can be life-threatening. Early diagnosis and What causes vasculitis? treatment is essential for the best chances There’s no single cause of vasculitis, of avoiding permanent damage to tissues and in most cases the exact cause is and organs. Most types respond well to unknown. We know that vasculitis isn’t treatment, and for many of them you’re directly inherited through the genes we get likely to make a full recovery, although from our parents, but genetic factors do relapses in the future are possible. play a part as several cases can occur in the The outcome depends on the type of same family. Genes could make you more vasculitis and how it affects you. Overall, likely to develop the condition, in which the best way to learn more about what case it may only take a small trigger (such might happen to you in the future is to as an infection or drugs) to start this off. talk to your doctor or another healthcare professional. We also know that some types of vasculitis – for example, those affecting the small blood vessels – can be related How is vasculitis to infections, particularly those associated with hepatitis. Some cases of vasculitis diagnosed? happen after certain drugs have been If you think you may be developing used, for example propylthiouracil vasculitis, you must see your doctor as (used to treat thyroid disease) and soon as possible. Infections, drugs and allopurinol (used to treat gout), as well as some foods can sometimes cause non-steroidal anti-inflammatory drugs vasculitis, so your doctor will probably (NSAIDs) and antibiotics. ask about the medications you’ve been taking and your general health during It’s thought that most forms of vasculitis the past few weeks. are a type of .

Page 17 of 32 Vasculitis information booklet Treatments are given in stages to keep your vasculitis under A full blood count can help to find out whether you have anaemia and whether control. you have normal levels of white blood cells (which fight infections) and (which are involved in clotting). Blood tests for antineutrophil cytoplasmic antibodies (ANCA) are important in the diagnosis of some types of vasculitis, particularly granulomatosis What tests are there? with polyangiitis, eosinophilic granulomatosis with polyangiitis and There are many tests that may be done microscopic polyangiitis. to help diagnose the condition. In this section we’ll look at the most common, A urea and electrolytes (U&E) test, an but there are others that you may need. estimated glomerular filtration rate (eGFR) Ask your rheumatologist, other specialist test or a creatinine test may be used to or GP if you’re not sure about what a check how your kidneys are working. Liver certain test will involve. function tests may also be carried out to check how your liver is working. Blood tests may be used to measure inflammation, for example: If you have vasculitis along with other • erythrocyte sedimentation rate (ESR) conditions, such as rheumatoid arthritis or lupus, then blood tests might be used • C-reactive protein (CRP).

Page 18 of 32 Vasculitis information booklet

to assess how active these other diseases • An angiogram is often done where are. Blood tests can measure the level abdominal organs such as the kidney of rheumatoid factor in rheumatoid and gut are involved. This involves arthritis, or the levels of complement injecting dye into the arteries so that (an enzyme system or group of proteins they show up on an x-ray. They can also in the blood) and antibodies in lupus. be done in Takayasu arteritis and giant Blood tests may be repeated from time cell arteritis to see how much the large to time to check how your condition blood vessels are involved. is responding to treatment. Other tests may be carried out to see how What treatments are the affected body organs are working – for example: there for vasculitis? The treatments used for vasculitis will • Urine tests will show the presence of depend upon which blood vessels and blood and/or protein, which are often organs are affected, as well as how much the first signs of an inflamed kidney. body tissue is affected. If the vasculitis People with eosinophilic granulomatosis only affects the skin, it may be enough with polyangiitis, granulomatosis with to treat any underlying infection or polyangiitis or microscopic polyangiitis to remove the drug that triggered the will have regular urine tests for blood vasculitis. However, in most cases, drug and protein. treatment will be needed to control the • X-rays, CT and MRI scans can be used disease and its symptoms and to stop or to check for chest problems. limit the damage caused by vasculitis. • Echocardiograms and electrocardiograms (ECGs) can be Drugs The two main types of drug used used to assess the heart. to treat vasculitis are steroids and • A biopsy may be needed to confirm immunosuppressant agents. Both act to whether the kidneys, the muscles, dampen down the immune system to skin or lungs are affected by vasculitis. reduce the strength of its attack on the A small piece of tissue is removed from tissues of the body. the organ in question for examination If you have vasculitis affecting the large or testing in a laboratory. blood vessels, then you’ll probably be • An ear, nose and throat (ENT) given steroid tablets. These are very assessment may be needed for effective for GCA and Takayasu arteritis, people with granulomatosis with and often immunosuppressant agents polyangiitis who have symptoms in aren’t needed in these conditions. these parts of the body.

Page 19 of 32 Vasculitis varies from one person to the next, so it’s important that you understand your doctor’s advice clearly...

...and to make sure your treatment isn’t interrupted. Vasculitis information booklet

If you have vasculitis affecting small • Hepatitis-associated PAN can be and/or medium-sized blood vessels, treated with antiviral treatment and then you may only need a small dose of plasma exchange. steroids to control it. However, you may Plasma exchange (also known as blood be given a combination of steroids and washing) involves being connected to a immunosuppressive drugs, probably machine that your blood passes through over several years, especially if vasculitis before being returned to you so it can affects the internal organs. For many be cleaned of the factors causing the types of vasculitis, including those vasculitis. Only a few people with the affecting the kidney, lungs or other vital most severe types of vasculitis – for organs (especially if it involves both example who have very severe kidney or small and medium-sized blood vessels), disease – will need plasma exchange. your treatment will be given in stages This will be done in specialist centres. (see Figure 6). Possible side-effects of steroids include If you have vasculitis that mainly affects weight gain, indigestion, , medium-sized arteries, then other thinning of the skin and thinning of the treatments can help, depending on bones (). If high doses of the condition: steroids are given, then you’ll also be • Kawasaki disease can be treated given drugs like to help effectively with injections of prevent osteoporosis. immunoglobulin (a type of protein).

Figure 6: The stages of treatment for some types of vasculitis

Stage 1: Stage 2: Stage 3: Bringing you to Keeping you Following up and remission in remission withdrawing therapy

Aim: to get the disease Aim: to keep the disease Aim: to keep your under control. under control once it’s vasculitis under An immunosuppressive in remission. Drugs (for control while gradually drug (for example example , decreasing the amount ) may or of treatment. be used to dampen down mycophenolate) may be the immune system, used with steroids. which is attacking the blood vessels. Steroid tablets or injections will be used in this stage.

Page 21 of 32 Vasculitis information booklet Gentle exercise can help keep muscles and joints healthy. See Arthritis Research UK drug leaflets Azathioprine; Cyclophosphamide; Methotrexate; Mycophenolate; Rituximab.

Self-help and daily living See Arthritis Research UK booklet If you do need treatment then it’s very and drug leaflet Osteoporosis; important that you follow your doctor’s Steroid tablets. instructions carefully. It’s important to learn and understand as much as you In some types of vasculitis (such as can about your illness and the treatment granulomatosis with polyangiitis), an options; you can discuss alternative infection may trigger a relapse. You treatment options with your medical may therefore be given antibacterial team. Vasculitis varies from one person to drugs such as co-trimoxazole to protect the next and from one type of vasculitis against this. These drugs can also help to another. It’s important to speak to your to protect against the increased risk doctor or other healthcare professional of infection caused by the stronger about any new symptoms you may have. immunosuppressive drugs. Exercise As with all medications, there may Vasculitis can cause tiredness, and it’s sometimes be side-effects. important to rest when you need to. Cyclophosphamide, for example, However, you should also try to keep can cause bleeding from the bladder, muscles and joints healthy by exercising. hair thinning and an increased risk of Start gently and gradually increase the infection. Unfortunately there’s also amount of exercise you do. Include some a significant risk that it can reduce fertility in both men and women. Because of weight-bearing exercise (anything these risks, cyclophosphamide will be that involves walking or running), and stopped or exchanged for a different swimming is also recommended. Ask your immunosuppressive drug as soon as your doctor for advice on how much exercise vasculitis is controlled. This is usually you should expect to be able to do. azathioprine, but methotrexate or mycophenolate might be used instead. See Arthritis Research UK booklet Rituximab, a biological therapy given Keep moving. by intravenous infusion, can also help to encourage remission in some types of vasculitis. Page 22 of 32

Vasculitis information booklet

Diet and nutrition See Arthritis Research UK booklet You won’t usually need to keep to any special diet. A healthy, low-, nutritious Diet and arthritis. and balanced diet is important for everyone, but if you’re on steroids it’s Stop smoking particularly important because these Smoking makes the blood vessels become can increase your appetite and cause narrower inside and can make vasculitis weight gain. Try not to over eat, and cut symptoms worse. Smoking can also down on fatty and sugary foods and others which are particularly high in worsen lung inflammation. It’s therefore calories. Instead, eat lots of fresh fruit very important to try to stop smoking. and vegetables and starchy foods like This will also improve any symptoms of potatoes and wholemeal bread, pasta Raynaud’s phenomenon. If you want to and rice. stop smoking see your doctor who will be able to give you advice and help Drinking plenty of water is helpful. It’s about quitting. healthy for everyone not to drink more than the recommended units of alcohol Keep warm a day; 3-4 a day for men and 2-3 a day If your fingers or toes turn blue in for women. Having at least two alcohol- free days a week is advisable. If you’re response to the cold, this may be due to taking steroids then you’re more at risk Raynaud’s phenomenon. Wearing warm of osteoporosis; having plenty of calcium clothes, including warm socks and gloves, in your diet is important to help prevent should improve blood circulation to your this from developing. Foods that are good hands and feet by helping to keep the sources of calcium include tinned sardines blood vessels open. (with bones), skimmed milk, yoghurt and certain vegetables such as broccoli. Research and new developments In the past, some types of vasculitis were very serious diseases, especially if they affected both small and medium-sized Most forms of arteries. Advances in treatment over the last two decades have completely vasculitis can now changed this and most forms of vasculitis be controlled can now be controlled and sometimes completely cured. The new problem to and sometimes be faced is that some treatments have completely cured. unpleasant side-effects – some are almost

Page 24 of 32 Vasculitis information booklet

as damaging as the disease itself, though Glossary in different ways. Anaemia – a shortage of haemoglobin Researchers are currently trying to better (oxygen-carrying pigment) in the understand what causes vasculitis. blood which makes it more difficult for Comparing what happens in different the blood to carry oxygen around the countries may help to find the answer – body. Anaemia can be caused by some for example: rheumatic diseases such as rheumatoid • Takayasu arteritis (TA) is much more arthritis or lupus, or by a shortage of iron common in the Far East and in Africa in the diet. It can also be a side-effect than in the UK. of some drugs used to treat arthritis. • GCA is extremely rare in India but very Aneurysm – a balloon-shaped sac common in northern Europe. that bulges out of a blood vessel wall. Some diseases or infections can weaken • Granulomatosis with polyangiitis is the vessel wall, and the pressure of blood more common in northern Europe than causes the weakened section to balloon in southern Europe. outwards. The bigger the aneurysm • Microscopic polyangiitis is more gets, the greater the risk of it bursting, common in southern Europe than which can result in internal bleeding and northern Europe. other complications. Researchers at the University of Angiogram – a type of scan that looks Cambridge have received funding from inside the arteries in your heart to find Arthritis Research UK to carry out a large out where and how severe any narrowed genetic study to investigate whether areas are. It’s also known as a coronary common differences that we all carry in angiogram or a cardiac catheterisation. our genes might predispose some people Antibodies – blood proteins that form to developing a disease called ANCA- in response to germs, viruses or any other associated Vasculitis. substances that the body sees as foreign Other work also being carried out by or dangerous. The role of antibodies is to scientists at the University of Cambridge is attack these foreign substances and make looking to increase our understanding of them harmless. the role played by bacteria in the nose on Antineutrophil cytoplasmic antibodies the development of granulomatosis with (ANCA) – a group of antibodies which polyangiitis. It is hoped the research may attach themselves to molecules in enable clinicians to predict those patients white blood cells rather than to foreign with a high risk of disease recurrence. substances. They’re commonly found in blood tests for a number of autoimmune disorders such as vasculitis, although they can’t definitely confirm the diagnosis. Page 25 of 32 Vasculitis information booklet

Antiviral treatment – drugs used to treat Computerised tomography (CT) scan infections caused by particular viruses. – a type of scan that records images of Autoimmune disease – a disorder of sections or ‘slices’ of the body using x-rays. the body’s defence mechanism (immune These images are then transformed by system), in which antibodies and other a computer into cross-sectional pictures. components of the immune system attack C-reactive protein (CRP) – a protein the body’s own tissue rather than germs, found in the blood. The level of C-reactive viruses and other foreign substances. protein in the blood rises in response to Biological therapies – drugs that inflammation and a blood test for the reduce joint inflammation in people with protein can therefore be used as a measure rheumatoid arthritis and some other of inflammation or disease activity. inflammatory diseases. They work by Creatinine test – a urine or blood test targeting specific molecules involved in used to monitor the kidneys. Creatinine the inflammatory process and include is a waste product from your muscles anti-TNF drugs (adalimumab, etanercept that’s processed by your kidneys, so by and infliximab) and rituximab. checking the levels of creatinine a doctor Biopsy – the removal of a small amount can tell how well your kidneys are working. of living tissue from your body. The Dialysis – a method of separating particles sample can help diagnose illness when in a liquid by passing them through examined under a microscope. a membrane. In kidney dialysis the blood Bisphosphonates – drugs used to is circulated through a special machine prevent the loss of bone mass and treat that uses this method to remove waste bone disorders such as osteoporosis and materials or poisons from the blood. Paget’s disease. They work by reducing Echocardiogram – a type of scan that high levels of calcium in the blood and uses waves to create detailed by slowing down bone metabolism. pictures of the inside of the heart. Complement – an enzyme ‘system’ This test helps show the structure and in the blood. An enzyme is a substance movement of the heart. that speeds up a biological reaction. Electrocardiogram (ECG) – a test Complement consists of at least 19 that records the electrical activity separate proteins and plays an important of the heart using a machine called an part in the body’s immune system. electrocardiograph. The aim of an ECG It allows foreign particles or micro- is to detect unusual heart rhythms and organisms to be made harmless, but also to identify heart problems. generates inflammation. Blood tests can – a type of show how much of each of the major which is able to absorb foreign matter. elements of complement is present. These cells are involved in allergic responses in the body. Page 26 of 32 Vasculitis information booklet

Erythrocyte sedimentation rate (ESR) – the immune system attacks the body’s a test that shows the level of inflammation own tissues. in the body and can help in the diagnosis Inflammation – a normal reaction of rheumatoid arthritis. Blood is separated to injury or infection of living tissues. in a machine with a rapidly rotating The flow of blood increases, resulting in container (a centrifuge), then left to stand heat and redness in the affected tissues, in a test tube. The ESR test measures and fluid and cells leak into the tissue, the speed at which the red blood cells causing swelling. (erythrocytes) settle. Infusion – a procedure where fluid is Estimated glomerular filtration rate transferred directly into a through a (eGFR) test – a test which estimates the thin plastic tube (cannula) over a period volume of blood filtered by your kidneys of hours or days. Intravenous infusions are over a given period of time to assess how used to transfer blood, fluids and essential well your kidneys are working. salts. It’s commonly known as a drip. Fatigue – a feeling of weariness that’s – blood tests used more extreme than simple tiredness. It to check the healthy functioning of can affect you physically, but it can also the liver. They’re frequently performed affect your concentration and motivation, to monitor drug treatment. and often comes on for no apparent reason and without warning. Lupus (systemic lupus erythematosus or SLE) – an autoimmune disease in Gout – an inflammatory arthritis caused which the immune system attacks the by a reaction to the formation of urate body’s own tissues. It can affect the skin, crystals in the joint. Gout comes and hair and joints and may also affect internal goes in severe flare-ups at first, but if organs. It’s often linked to a condition not treated it can eventually lead to joint called antiphospholipid syndrome (APS). damage. It often affects the big toe. Magnetic resonance imaging (MRI) Hepatitis – inflammation of the liver. scan – a type of scan that uses high- This can cause yellowing of the skin frequency radio waves in a strong (jaundice), fever, abdominal (tummy) pain magnetic field to build up pictures of the and an enlarged liver. inside of the body. It works by detecting Immunoglobulins – a class of blood water molecules in the body’s tissue that proteins that are responsible for immunity give out a characteristic signal in the to specific infections. magnetic field. An MRI scan can show up Immunosuppressive drugs – drugs soft-tissue structures as well as bones. that suppress the actions of the immune Mucous membrane – the type of system. They’re often used in conditions membrane that lines areas of the body such as rheumatoid arthritis where such as the mouth, nasal passages,

Page 27 of 32 Vasculitis information booklet

stomach and gut, vagina, and passages other conditions. However the presence to the lungs. of rheumatoid factor can’t definitely Osteoporosis – a condition where confirm the diagnosis. bones become less dense and more Sjögren’s syndrome – an autoimmune fragile, which means they break or disorder that’s characterised by dry eyes fracture more easily. and/or a dry mouth, aching and fatigue. Plasma exchange – a procedure Steroids – drugs that have a very to separate blood into its different powerful effect on inflammation. parts and remove the plasma and the The adrenal glands in the body produce it contains which cause a natural supply but much larger doses disease. Plasma from a donor or a plasma are used to treat autoimmune diseases. substitute is then returned in its place. is the most commonly Polymyalgia rheumatica (PMR) – a used steroid. rheumatic condition in which you have Urea and electrolytes (U&E) test – many (poly) painful muscles (). a blood test that mainly monitors the It’s characterised by pain and stiffness of kidneys. This is frequently carried out to the muscles of the neck, hips, shoulders monitor drug treatment. and thighs, which is usually worse in the mornings. Raynaud’s phenomenon – a circulatory Where can I find out more? problem that causes the blood supply If you’ve found this information useful to certain parts of the body to be greatly you might be interested in these other reduced. It can make the fingers and titles from our range: toes go temporarily cold and numb and Conditions they turn white, then blue, then red. • Giant cell arteritis (temporal arteritis) Raynaud’s phenomenon can also occur Gout with the condition . • Lupus (SLE) Rheumatoid arthritis – an inflammatory • disease affecting the joints, particularly • Polymyalgia rheumatica (PMR) the lining of the joint. It most commonly • Raynaud’s phenomenon starts in the smaller joints in a symmetrical pattern – that is, for example, • Rheumatoid arthritis in both hands or both wrists at once. • Sjögren’s syndrome Rheumatoid factor – a blood protein Self-help and daily living produced by a reaction in the immune • Diet and arthritis system. About 80% of people with rheumatoid arthritis test positive for this • Keep moving protein, and it can be present in some Page 28 of 32 Vasculitis information booklet

Drug leaflets PMRGCAUK (Polymyalgia Rheumatica • Azathioprine and Giant Cell Arteritis UK) London WC1N 3XX • Cyclophosphamide Phone: 0300 111 5090 • Drugs and arthritis Email: [email protected] • Local steroid injections www.pmrgcauk.com • Methotrexate National Kidney Federation The Point, Coach Road • Mycophenolate Shireoaks, Worksop • Rituximab Notts S81 8BW • Steroid tablets Helpline: 0845 6010 209 www.kidney.org.uk You can download all of our booklets and leaflets from our website or order Vasculitis Foundation them by contacting: PO Box 28660 Kansas City Arthritis Research UK MO 64188-8660 Copeman USA St Mary’s Court www.vasculitisfoundation.org St Mary’s Gate, Chesterfield Derbyshire S41 7TD Vasculitis UK Phone: 0300 790 0400 John Mills, West Bank House www.arthritisresearchuk.org Winster, Matlock Derbyshire DE4 2DQ Related organisations Helpline: 0300 365 0075 The following organisations may Email: [email protected] be able to provide additional advice www.vasculitis.org.uk and information: Vasculitis UK also produces the Vasculitis Arthritis Care Roadmap, which is a guide covering Floor 4, Linen Court every aspect of the condition. You can 10 East Road download it from London N1 6AD www.vasculitis.org.uk/about/routemap Phone: 020 7380 6500 Links to sites and resources provided Helpline: 0808 800 4050 by third parties are provided for your Email: [email protected] general information only. We have no www.arthritiscare.org.uk control over the contents of those sites Churg–Strauss Syndrome or resources and we give no warranty Association (USA) about their accuracy or suitability. www.cssassociation.org You should always consult with your GP or other medical professional. Page 29 of 32 Vasculitis information booklet

We’re here to help Arthritis Research UK is the charity funding and giving insight into the latest leading the fight against arthritis. treatment and self-help available. We fund scientific and medical We often feature case studies and research into all types of arthritis and have regular columns for questions musculoskeletal conditions. and answers, as well as readers’ hints We’re working to take the pain away and tips for managing arthritis. for sufferers with all forms of arthritis and helping people to remain active. We’ll do this by funding high-quality Tell us what you think research, providing information Please send your views to: and campaigning. bookletfeedback@ Everything we do is underpinned arthritisresearchuk.org by research. or write to us at: Arthritis Research UK, Copeman We publish over 60 information booklets House, St Mary’s Court, St Mary’s Gate, which help people affected by arthritis Chesterfield, Derbyshire S41 7TD to understand more about the condition, its treatment, therapies and how to help themselves. A team of people contributed to this booklet. We also produce a range of separate The original text was written by Dr Richard leaflets on many of the drugs used Watts, who has expertise in the subject. It was for arthritis and related conditions. assessed at draft stage by rheumatology We recommend that you read the nursing lecturer Dr Janice Mooney, relevant leaflet for more detailed rheumatology nurse practitioner Karina Blunn information about your medication. and chairman of Vasculitis UK John Mills. An Arthritis Research UK editor revised the Please also let us know if you’d like text to make it easy to understand. to receive an email alert for our An Arthritis Research UK medical advisor, quarterly online magazine, Arthritis Dr Luke Gompels, is responsible for the Today, which keeps you up to date with content overall. current research and education news, highlighting key projects that we’re

Page 30 of 32 Get involved You can help to take the pain away from millions of people in the UK by:

• volunteering • supporting our campaigns • taking part in a fundraising event • making a donation • asking your company to support us • buying products from our online and high-street shops.

To get more actively involved, please call us on 0300 790 0400, email us at [email protected] or go to www.arthritisresearchuk.org Arthritis Research UK Copeman House St Mary’s Court St Mary’s Gate, Chesterfield Derbyshire S41 7TD Tel 0300 790 0400 calls charged at standard rate www.arthritisresearchuk.org

Registered Charity England and Wales No 207711, Scotland SC041156 © Arthritis Research UK 2011 Published January 2016 2047/VASC/16-1 Printed on material from responsible sources