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 most people the inflamed wall slowly thickens and the artery narrows, making it harder for blood to travel through. The narrowing develops gradually, the arteries do not usually block completely, and a dangerous loss of blood supply to the limbs or major organs is unusual. It is this slow narrowing, and the weak or absent wrist pulse it can cause, that gave the condition its older name of pulseless disease.

Takayasu arteritis is very rare in the UK, with only around 100 new cases a year, and is more common in the Far East and Africa. It mostly begins between the ages of 5 and 40, 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 and a reduced appetite
• 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 an infection, stress or anaemia, and the diagnosis is often reached only when these threads are 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 work well. 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. Where the disease stays active despite these, biologic medicines that block interleukin-6, tocilizumab or sarilumab, are used.

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, and most people live full lives around their monitoring.

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.

Because the condition is rare, care is often shared with one of the specialist vasculitis centres, particularly where surgery is being considered. That is a strength of the system rather than a sign of trouble: it means decisions about a rare disease are made by teams who see it regularly.

Most of what helps is the steady, unglamorous kind of self-care. Take the medicines as prescribed, and never stop steroid tablets suddenly. Keep up the monitoring appointments and scans even when you feel completely well, because feeling well and the arteries being quiet are not always the same thing. Staying active, eating well and looking after your general heart health all support the arteries the condition cares most about, and if you smoke, stopping is the single most useful change you can make for your blood vessels.

It also helps to keep a simple record of your symptoms, because with a relapsing condition the pattern over months tells your team more than any single appointment can.

• Contact your rheumatology team if the symptoms that led to your diagnosis return, if you develop new arm or leg pain with use, or if you feel generally unwell with fevers or night sweats again: these can signal returning inflammation that needs review.
• Call 999 if chest pain is crushing, tight or pressure-like, spreads to an arm, the jaw, the neck or the back, or comes with breathlessness, sweating or feeling sick.
• Call 999 for sudden weakness or numbness on one side, a drooping face, or sudden difficulty speaking, which are signs of a possible stroke.
• Seek same-day advice from your GP or NHS 111 if you feel rapidly or increasingly unwell in yourself.

Takayasu arteritis is rare, and its early symptoms are common: tiredness, fevers, aches and feeling run down have many more ordinary explanations. 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 most people live with rather than around.

Is Takayasu arteritis serious?

It is a condition to take seriously, and also one that is treatable. The inflammation narrows the affected arteries slowly, they do not usually block completely, and a dangerous loss of blood supply to the limbs or major organs is unusual. Treatment settles the inflammation and protects the arteries, and with modern care 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: the first symptoms usually appear between the ages of 5 and 40, and the large majority of those affected, around eight or nine in ten, are women. It is very rare in the UK, with only around 100 new cases a year, and is more common in the Far East and 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 behave like age-separated siblings. 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 respond well to steroids, 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, biologic medicines that block interleukin-6, tocilizumab or sarilumab, are used. 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: typically a set at diagnosis and then further imaging at regular intervals. This is because blood tests alone cannot always show what the artery walls are doing, so imaging does some of the watching that blood tests do in other conditions. 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.