Takayasu arteritis

Takayasu arteritis is one of the large-vessel vasculitis family, the group of conditions in which the immune system inflames the walls of the body's biggest arteries. Here the target is the aorta, the main artery leaving the heart, together with its major branches, including the arteries to the head and neck, the arms, the heart and the kidneys. Why the immune system does this is not fully understood.

The inflammation matters because of what it does to the artery wall over time. In around nine in ten people the wall thickens until it narrows or blocks one or more of the affected arteries, making it harder for blood to travel through, and in around a quarter, a weakened stretch of an artery wall swells outwards into a bulge called an aneurysm. The wall changes are slow to build rather than sudden, which is exactly why early control of the inflammation, and regular review of the arteries, matter so much. The condition's older name, pulseless disease, comes from that narrowing and the faint or missing pulse at the wrist it can produce.

Takayasu arteritis is very rare in the UK, with only around 100 new cases a year, and is more common in East Asia, South Asia and Africa. It mostly begins between the ages of 5 and 40, most often in the twenties and thirties, and around eight or nine in ten of those affected are women.

Takayasu arteritis tends to show itself in two ways, often at different stages.

Early on, the symptoms are usually general rather than pointing at the arteries:

• Profound tiredness and a general sense of being unwell
• Fever or night sweats
• Weight loss
• Aching muscles and painful joints
• Sometimes a rash

Some people also notice pain and tenderness over the front of the neck, where the carotid arteries run; this affects roughly a quarter of people with the condition.

Later, symptoms can come from the narrowing itself, most often painful, numb or cold arms or legs, particularly with use. A doctor may find a weak or absent pulse at one wrist. Because the early picture is so general, it is easily put down to something more ordinary, and the diagnosis often comes late, once these threads are finally looked at together.

There is no single test. The diagnosis brings together the pattern of symptoms, an examination that includes checking the pulses, and blood tests, which typically show raised inflammatory markers while the disease is active. Doctors use formal criteria to make the diagnosis with confidence.

Imaging is central, because it shows the artery walls directly. Depending on the situation this can include high-resolution ultrasound, magnetic resonance angiography (MRA), CT angiography and PET scanning, which between them show where the inflammation is, how the vessel walls have changed and how the blood is flowing. The same kinds of imaging are then used to keep an eye on the arteries during follow-up.

Treatment aims to settle the inflammation, protect the arteries and then keep the disease quiet with as little medication as possible.

Steroid tablets, usually prednisolone, are the first treatment, and around half of people need a second medicine alongside them to bring the inflammation under control. A steroid-sparing medicine that calms the immune system, commonly methotrexate, azathioprine or mycophenolate, is often used alongside so the steroid dose can be reduced without the inflammation returning; our guide to steroids in rheumatology explains how steroid courses are managed and why they should never be stopped suddenly. Biologic treatment is not usually the next move if the disease stays active on those; first comes another immune-suppressing medicine. When the inflammation still refuses to settle, the biologic tocilizumab can be added; it blocks the action of a messenger called interleukin-6, and at this stage it is routinely funded for Takayasu arteritis in England. Tumour necrosis factor is a different messenger, and a medicine that blocks it is what some specialists reach for instead; which is used depends on the individual and on the centre giving the care.

Occasionally, a narrowed artery causes enough trouble to need a planned procedure to restore the blood flow. Where that is needed, it is carried out at a specialist centre, and timed for when the inflammation is quiet, because operating on an inflamed artery gives poorer results.

For about one person in five, Takayasu arteritis is a single episode that settles and does not return. More typically it follows a relapsing and remitting course, with quiet periods and occasional returns of inflammation that need treatment to be stepped up for a while.

Because of that, everyone with the condition has long-term follow-up. Blood tests help, but they cannot always show what the artery walls are doing, so imaging carries part of the load: typically a set of scans at diagnosis and then further imaging at regular intervals. Picking up change early, before it causes symptoms, is the whole point of the follow-up, and it is a large part of why the modern outlook is good. Long-term survival in the UK and USA is up to 98 per cent at ten years. The heart and the blood vessels do stay at more risk than average, which is what the monitoring, and the care taken over blood pressure and cholesterol, are for, and many people carry on with their lives around that follow-up.

Takayasu arteritis is a rheumatology-led condition, and it is also one that benefits from teamwork. The medicines, monitoring and overall plan sit with the rheumatologist; vascular specialists join when an artery needs a procedure; and where the arteries to the heart are involved, cardiology shares the care. Our guide to inflammation and your heart explains more generally how inflammatory conditions and the heart are looked after together. Dr Borukhson practises within a world-renowned tertiary centre, with ready access to consultant colleagues in the other specialties this condition may call on. Where it helps, she can bring those specialists in directly, and particularly complex cases can go to a multidisciplinary team meeting (MDT) with minimal delay. Whenever more than one kind of expertise is needed, the strands of your care can be drawn together and decisions reached promptly.

Because the condition is rare, care is often shared with one of the specialist vasculitis centres, particularly where surgery is being considered. Shared care is normal for a condition this uncommon: it means decisions are made by teams who see it regularly.

Living well with Takayasu arteritis is mostly a matter of routine. Keep to your prescribed medicines, and never stop steroid tablets suddenly, however well you feel. Come to the monitoring appointments and scans even during the quiet spells, because an artery can stay inflamed without giving you any warning of it. Staying active, eating well and keeping your blood pressure and cholesterol in check all protect the arteries this condition targets, and if you smoke, stopping is the single most useful change you can make for your blood vessels.

A simple symptom record is worth keeping too. Takayasu arteritis comes and goes, and the shape of the past few months is often far more revealing than one clinic visit on its own.

If a pregnancy is something you are planning, talk it through with your specialist first: many women with Takayasu arteritis have successful pregnancies, but blood pressure can climb to a dangerous level in pregnancy, some of its medicines must be swapped before you try, and the disease is best quiet; our guide to arthritis, autoimmune conditions and pregnancy has the medicines detail.

Takayasu arteritis is followed closely, and most changes can wait for the next planned review; a few cannot.

Treat any of these as a 999 or A&E emergency: call 999 and do not drive yourself to A&E.

• Severe illness with suspected sepsis: a high fever with shaking, confusion, rapid breathing, or feeling dramatically worse over hours
• With a fever or feeling unwell, a rash that does not fade when you press it, a stiff neck with eyes that cannot bear bright light, or skin, lips or tongue turning blue, grey, pale or blotchy, since these can be signs of meningitis or sepsis; on black or brown skin the rash and the change of colour may be easier to see on the palms of the hands or the soles of the feet
• Chest pain that is crushing, tight or like a pressure, that spreads to an arm, the jaw, the neck, the stomach or the back, that comes with breathlessness, sweating, feeling sick or feeling light-headed, or that came on suddenly and does not go away
• Breathlessness that is severe, with gasping, choking or no breath to get words out; or coughing up blood, since the heart and the arteries to the lungs can both be involved
• Palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, since a heart whose muscle or rhythm is affected has to be checked at once
• Sudden severe pain in the abdomen or back, since a weakened, bulging stretch of the aorta can tear or leak
• A blackout, since the arteries to the head and the heart's own supply can both be affected
• An arm or leg that has suddenly gone cold, pale, numb or very painful and stays so, since an artery to the limb can block
• A rash that spreads across the body, skin that blisters or peels, or lips, mouth or eyes that turn sore or swollen, since this can be a severe reaction to a medicine, such as Stevens-Johnson syndrome, which needs treating in hospital straight away
• Sudden weakness or numbness on one side, a drooping face or difficulty speaking, a sudden severe headache, sudden confusion or a seizure, or sudden loss of vision, any of which can be a stroke, even if it stops after a short time, and so can new double vision or sight that blurs suddenly, since in Takayasu the arteries supplying the brain and the eyes can narrow or block
• An eye that suddenly and completely loses its sight
• Vomiting that will not let your tablets stay down, or, during an illness or after an injury, becoming very drowsy or confused, or fainting, after more than a few weeks on steroid tablets, or repeated courses or injections in the past year, since your body may be running short of cortisol and the steroid may need to be given by injection; have your NHS Steroid Emergency Card ready
• Lips, mouth, throat or tongue that suddenly swell, a throat that feels tight, very fast breathing or a struggle to breathe or to swallow, skin, tongue or lips turning blue, grey or pale (on black or brown skin this may be easier to see on the palms of the hands or the soles of the feet), becoming suddenly very confused, drowsy or dizzy, or fainting and not being able to be woken, since this can be a severe allergic reaction, anaphylaxis, which can follow a dose of any medicine
• Vomiting blood, or bleeding from the back passage that is heavy or will not stop, most of all with faintness, confusion or pain in the abdomen, or vomit that is black or dark brown, since a stomach ulcer on prednisolone can bleed
• Serious harm you have already done to yourself, such as an overdose, or a feeling that you may be about to harm yourself, since you need help to keep you safe now

Any of these needs an assessment that is urgent, on the same day, from your GP, your rheumatology team or NHS 111:

• A fever, or feeling generally unwell, on immunosuppressant, biologic or steroid treatment, since an infection has to be treated early
• A joint that is hot and swollen, with a fever or feeling unwell, since an infected joint has to be ruled out at once, at A&E or through NHS 111
• A joint that has become hot and very swollen suddenly, or is worsening quickly, even without a fever, since an infected joint does not always announce itself with one; a joint that will take no weight at all, or that cannot be moved, is a 999 or A&E emergency, whether or not there is a fever
• New chest pain, new palpitations, or breathlessness that is new or getting worse; palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, belong to the first list; in pregnancy, breathlessness that comes on suddenly, or when you lie down, or with dizziness, is for your midwife or maternity unit straight away, as the pregnancy line below says
• A blood pressure reading far above your usual, since the arteries to the kidneys can narrow and push the pressure up
• Sight that has blurred or dimmed, or that is lost in part, which needs an eye casualty or urgent eye clinic the same day, since in Takayasu the arteries that feed the eyes can narrow; sight that blurs or is lost suddenly, in part or wholly, even for a short time, belongs to the first list, in pregnancy too; in pregnancy, any change in sight, blurring or flashing lights among them, is also for your midwife or maternity unit straight away, as the pregnancy line below says, since it can be a sign of pre-eclampsia
• Thoughts of ending your life or of hurting yourself, or harm you have done to yourself that is not serious, short of the line in the first list, which steroids can bring on: ask your GP to see you that day or call NHS 111, and Samaritans answer at any hour on 116 123
• A marked change in mood or behaviour, confusion, or seeing or hearing things that are not there, on steroid treatment, since prednisolone can do this, the more so the higher the dose; confusion that comes on suddenly is a 999 or A&E emergency, as the first list says, and so, after more than a few weeks on steroid tablets, or repeated courses or injections in the past year, is becoming very drowsy or confused during an illness or after an injury
• Unusual thirst or hunger, passing urine more often, or feeling sleepy or confused, on steroids, or swelling of the arms or legs, bruising or bleeding that is not normal, black or dark red stools, severe stomach pain, severe back pain, a severe upset stomach, being sick, or very bad dizziness, since each can be a steroid effect that needs checking without delay; pain in the abdomen or back that comes on suddenly and severely is a 999 or A&E emergency, as the first list says, and so, after more than a few weeks on steroid tablets, or repeated courses or injections in the past year, is vomiting that will not let your tablets stay down, or, during an illness or after an injury, becoming very drowsy or confused, or fainting; in pregnancy, bleeding that is not normal, and stomach pain that is severe, belong to the pregnancy line below
• Contact with measles, chickenpox or shingles, since these infections can be severe on steroids and a medicine to protect you may be needed
• Being near someone with measles, chickenpox or shingles, while you take a medicine that weakens the immune system, since measles and chickenpox can be dangerous when the immune system is weakened, and you can catch chickenpox from someone with shingles if you have never had chickenpox; NHS 111 can tell you what to do that day
• In pregnancy, any bleeding, stomach pain that is severe or does not go away after a rest, reduced baby movements, a severe headache or visual disturbance, sudden swelling of the face, hands, legs or feet, or breathlessness that comes on suddenly, or when you lie down, or with dizziness: contact your midwife or maternity unit straight away, whatever the time; bleeding that is heavy, or that comes with severe pain in the tummy, pain in the shoulder, or feeling sick, faint or dizzy, or passing out, is a 999 or A&E emergency: call 999 and do not drive yourself to A&E; pain in the abdomen or back that comes on suddenly and severely is a 999 or A&E emergency, as the first list says
• A high temperature in pregnancy, whatever medicines you are taking, since a fever can be a sign of an infection that needs checking; your midwife or maternity unit can advise
• In pregnancy, contact with someone who has an illness with a rash, measles, chickenpox and shingles among them, whatever medicines you are taking, since some of these infections can harm an unborn baby: ring your midwife or maternity unit, or your GP, straight away

Some things belong with your rheumatology team promptly, without waiting for the next appointment: new pain in an arm or a leg when you use it, tiredness or night sweats creeping back, or any return of the symptoms that were there at the start, since each can mean the inflammation has woken again and is easier to settle early (a fever while you are on treatment belongs to the same-day list above, and with the signs of sepsis in the first list it is a 999 or A&E emergency). Medicine questions, and the uneventful months between one scan and the next, keep for the planned reviews. If you take methotrexate, a steroid or a biologic, the warning signs that belong to each are set out in our guides to methotrexate, DMARDs, steroids and biologics and JAK inhibitors. If you cannot tell how urgent something is, asking is never a mistake.

Takayasu arteritis is rare, and its early symptoms, tiredness, fevers, aches and feeling run down, are the ordinary ones that usually have another explanation. What deserves a specialist eye is the combination and the persistence, especially in a younger woman, and even more so with neck tenderness, limb pain on use or a pulse that seems to have gone missing. An accurate diagnosis, made with modern imaging and confirmed against formal criteria, opens the door to treatment that settles the inflammation and to the long-term follow-up that protects the arteries. Caught early and monitored well, this is a condition many people live a full life with.

Is Takayasu arteritis serious?

It is a condition to take seriously, and also one that is treatable. As the inflammation goes on, the walls of the affected arteries thicken: in around nine in ten people an artery ends up narrowed or blocked, and in around a quarter, part of an artery wall weakens and bulges outwards, the swelling known as an aneurysm. Because those changes come on slowly rather than all at once, early treatment and regular monitoring work well against them: calming the inflammation protects the arteries, and with modern care, and the monitoring that also keeps the heart and blood vessels protected, the long-term outlook is good. The keys are an accurate diagnosis and regular follow-up, both of which this guide explains.

Who gets Takayasu arteritis?

Mostly younger people: it usually starts somewhere between 5 and 40, the twenties and thirties above all, and eight or nine in every ten of those affected are women. In the UK it is very rare, about 100 new cases a year, and it is seen more often in East and South Asia and in Africa. Because it is rare and its early symptoms are so general, it is often not the first explanation anyone thinks of, which is why the diagnosis can take time.

Why is it sometimes called pulseless disease?

Because of what the inflammation does to the arteries. In most people the inflamed artery walls slowly thicken and narrow, and when the artery supplying an arm is affected, the pulse at the wrist can become weak or impossible to feel. That striking examination finding gave the condition its older name, and it is one of the clues that can point a doctor towards the diagnosis.

How is Takayasu arteritis different from giant cell arteritis?

They are the two main forms of large-vessel vasculitis, and they differ mainly in the age at which they begin. Giant cell arteritis affects older adults, typically over 50, and is best known for involving the arteries around the temples. Takayasu arteritis begins in younger people, typically under 50 and often much younger, and centres on the aorta and its main branches. Both are treated with steroids first, in Takayasu arteritis often with a second medicine alongside and in giant cell arteritis sometimes, and their assessment and monitoring use similar imaging.

How is it treated?

Treatment starts with steroid tablets, usually prednisolone, to settle the inflammation, and often a steroid-sparing medicine such as methotrexate, azathioprine or mycophenolate alongside so the steroid dose can be brought down. Where the disease keeps rumbling despite these, the usual next step is a further immune-suppressing medicine, and if the inflammation still will not settle, a biologic can be added: tocilizumab, which blocks the action of a messenger called interleukin-6, is routinely funded for Takayasu arteritis in England at that stage, though some specialists turn instead to a medicine that blocks tumour necrosis factor. Occasionally a narrowed artery needs a planned procedure, which is carried out at a specialist centre once the inflammation is quiet.

Will I need scans for the rest of my life?

Long-term follow-up, yes, and scans are a normal part of it: scans at the start, then at intervals the team sets. The artery wall can change without the blood tests showing it, which is why the scans carry part of the monitoring here. It is best seen as protection rather than a warning sign: regular monitoring is how any change is picked up early and treated before it matters.