Takayasu arteritis: a rare large-vessel vasculitis of the aorta and its branches

The NHS explains that vasculitis is a group of conditions that cause inflammation of the blood vessels, in which the immune system attacks healthy blood vessels, causing them to become swollen and narrow. Takayasu arteritis is one of the large vessel types. Versus Arthritis describes it as an inflammatory disease that affects the main artery from the heart (the aorta) and its large branches, usually in younger women.

It is rare in the UK. Versus Arthritis puts it at only 100 new cases a year, and notes that it is more common in the Far East and Africa and tends to affect younger Asian women. The NHS notes that it is very rare in the UK. An NHS England consultation document from 2016 quotes an estimated incidence of 0.7 to 1.5 per million per year in Europe and an average age of onset of 23 years, which is consistent with it being a condition of young adults.

The aorta and its branches supply the arms, head, kidneys and heart. Versus Arthritis lists the carotid arteries in the head and brain, the coronary arteries, the renal arteries to the kidneys and the arteries that take blood to the arms as vessels that may be affected. The name pulseless disease reflects the weak or absent pulses that can result.

The NHS lists extreme tiredness, a high temperature, weight loss, muscle and joint pain, and painful, numb or cold limbs as the symptoms of Takayasu arteritis. Early symptoms can resemble many other illnesses, which is one reason for delay in diagnosis.

As the arteries narrow, symptoms relate to reduced blood flow to a particular area. Examples include arm fatigue or pain on use, differences in pulse or blood pressure between the arms, headaches, dizziness or visual symptoms, high blood pressure from narrowed kidney arteries, and chest pain or breathlessness. Versus Arthritis explains that narrowing develops slowly and arteries do not usually block completely, so symptoms are often gradual rather than sudden.

The NHS England document describes the pattern in more detail: in 90% of patients one or more arteries become narrowed or blocked, hence the name pulseless disease, and in 25% part of an artery may swell to form an aneurysm. In some patients widening of the aorta can cause the aortic valve to fail and need replacement.

There is no single test. Versus Arthritis explains that for vasculitis in general blood tests may measure inflammation (ESR or CRP), that scans such as X-ray, CT and MRI are used, and that imaging can be used in Takayasu arteritis and giant cell arteritis to see how much the large blood vessels are involved. A rheumatologist usually leads diagnosis, sometimes with vascular surgeons, cardiologists or other specialists.

The NHS England document says that everyone with Takayasu arteritis should be reviewed at regular intervals to formally assess disease activity and damage using a formal instrument, predominantly with non-invasive imaging, so that remission, resistant disease or relapse can be documented accurately. Because narrowing can affect blood flow to the arms, doctors commonly compare blood pressure and pulses in both arms.

The NHS says Takayasu arteritis is usually treated with steroid medicine. Versus Arthritis says that for vasculitis affecting the large blood vessels you will probably be given steroid tablets, which are very effective for giant cell arteritis and Takayasu arteritis, and that immunosuppressive agents often are not needed for these conditions. Possible side effects of steroids include weight gain, indigestion, diabetes, thinning of the skin and thinning of the bones, and when high doses are used drugs such as bisphosphonates may be given to protect bone.

The NHS England consultation document says treatment involves three phases: remission induction, remission maintenance and treatment of relapse. It notes that relapse is common, that steroids and immunosuppressants have limited effectiveness for some people, and that consensus clinical opinion estimates that about 50% of patients develop treatment-resistant disease and may benefit from a biologic agent. It sets out a proposal to routinely commission tocilizumab, an interleukin-6 blocking antibody, for adults with Takayasu arteritis. As this was a 2016 draft for consultation, ask your specialist what is currently available on the NHS.

Versus Arthritis also describes stages of treatment used in many vasculitides: bringing the disease to remission, keeping it in remission with drugs such as azathioprine, methotrexate or mycophenolate with steroids, then following up and gradually withdrawing therapy while keeping disease under control.

The NHS England document says that without treatment Takayasu arteritis can lead to organ failure and to irreversible ischaemia from large-vessel narrowing or aneurysm that may require potentially hazardous large vessel reconstruction, and that relapse and poor response carry a risk of further ischaemic damage.

Where a narrowing is severe and is causing symptoms, a vascular team may consider procedures to restore blood flow, which the NHS England document refers to as large vessel reconstruction. Your vasculitis team and a vascular surgeon decide whether and when this is appropriate.

Versus Arthritis says most types of vasculitis respond well to treatment and for many types you are likely to make a full recovery, although relapses in the future are possible, and the outcome depends on the type and how it affects you. Early diagnosis and treatment is essential to avoid permanent tissue and organ damage.

In practical terms, long-term care usually includes regular blood pressure checks in both arms, monitoring of inflammation markers and periodic imaging, bone protection if you take steroids for a long time, and attention to cardiovascular risk factors such as smoking, cholesterol and diabetes. Versus Arthritis notes that if you have vasculitis you should have one consultant in overall charge of your care, with a multidisciplinary team where several organs are involved. Tell your team if you are planning a pregnancy, because disease control and medicines need to be reviewed beforehand.

Call 999 or go to A&E for sudden chest pain, sudden severe breathlessness, signs of stroke such as facial droop, arm weakness or slurred speech, sudden loss of vision, or a cold, pale, painful limb that has suddenly lost its pulse. Contact your specialist team promptly for new headaches, visual changes, dizziness, arm pain on use, a fever or a flare in your general symptoms, because these can signal active disease.

See a GP for unexplained tiredness with fever, weight loss and muscle or joint aches that persist, particularly in a younger adult, or for different blood pressure readings in each arm.