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Takayasu arteritis

This is part of Rare diseases.

Diagnosis: Takayasu arteritis

Synonyms: --

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Date of publication: 2014-10-16
Version: 2.0

ICD-10

M31.4

The disease

Takayasu arteritis (inflammation of the arterial walls) is a chronic disease affecting the major arteries. It affects blood vessels such as the aorta and its branches, which carry blood at high pressures. Walls of blood vessels become inflamed, leading to constrictions or a complete blockage. Circulatory abnormalities may cause strokes, pains in the arms and heart attacks.

The disease was named after Japanese ophthalmologist Mikito Takayasu, who was the first to describe it in the early 1900's.

Occurrence

In Sweden, it is believed that the occurrence of Takayasu arteritis is similar to the rest of Europe. Current studies in England show it affects 5 inhabitants per 100,000, meaning that there are fewer than 50 people with the disease in Sweden. The disease is more common in women than men, and found much more frequently in Asia.

Cause

The cause of the disease is unknown. Symptoms are caused by an inflammation in the walls of blood vessels, the result of an autoimmune reaction where the body attacks its own tissues. When examined under a microscope, inflamed tissue is shown to contain different types of white blood cells and it is also possible to establish the presence of various immunomodulating agents which cause inflammation (cytokines). When blood vessels become inflamed due to the presence of white blood cells their walls thicken, which may lead to blood vessels becoming constricted or totally blocked.

Inflammation of the walls of the blood vessel is characteristic of vasculitis. Forms of the disease are categorized in part according to the size of the blood vessels they affect and Takayasu arteritis is a form of large vessel vasculitis. It is important not to confuse different forms of vasculitis as they have different symptoms and require different treatments.

Heredity

Takayasu arteritis is not hereditary.

Symptoms

The disease usually presents before the age of 40, and also manifests in children. It is likely that it also occurs in older people but it is then difficult to distinguish from other forms of giant cell arteritis. Changes are usually found in the aorta, around the aortic bow near the heart and in vessels leading from the aorta to the arms and brain. Sometimes the disease is more widely spread and is also found in the abdominal aorta and the blood vessels leaving the kidneys. In a small group of people the disease may be confined to the abdominal aorta.

Circulatory problems may lead to pain in the arms, or to stroke or heart attack, depending on which blood vessels are affected. If the arteries leading to the kidneys become constricted, high blood pressure may result. If blood leaks between the left ventricle of the heart and the aorta as a result of damage to the valves, cardiac insufficiency may result. While blood vessels may become narrower, the aorta may become abnormally wide (aneurysm).

Active periods of inflammation may result in mild fever, fatigue and pain in the region of the inflamed blood vessels of the throat. Other symptoms include headache, pains in the joints and/or muscles, night sweats and loss of weight. Often, but not always, signs of inflammation include elevated ESR (erthrocyte sedimation rate) and CRP (C reactive protein) levels in the blood.

Symptoms indicating reduced blood supply to the brain include stroke and TIA's (transient ischaemic attacks) which involve temporary, short periods of dizziness and loss of vision. Insufficient blood supply to the muscles may cause short-term pain while the person is moving. When resting the pain disappears (claudicatio). Other signs of Takayasu arteritis include weak pulse in the arms, pale, cold hands and different blood pressure levels in the left and right arms as a result of the narrowing of specific, localized blood vessels.

The disease usually manifests in bouts, alternating between active periods and long periods of remission when activity is low or absent. In some people, symptoms are very mild. The disease does not need to limit the individual's ability to lead an active life.

Diagnosis

Takayasu arteritis is suspected if typical signs and symptoms present, especially in younger people and in cases where blood tests show signs of inflammation. Sometimes it may not be possible to feel a pulse in one or both wrists. In order to detect low blood pressure it is important to measure pressure in both arms to see if there is a difference, and to test blood pressure in the legs to see if there are differences between the upper and lower body. It is also important to listen for heart murmur at the throat, abdomen and over the heart.

It is often difficult to take tissue samples from inflamed blood vessels for microscopic analysis. For this reason, X-ray examinations of the aorta and its branches are often made. In the past, dye (contrast material) was injected directly into the arteries to see the contours of the interior of the blood vessel. Currently, CT (computed tomography) scans are often used instead. MRA (magnetic resonance angiography) scans, and sometimes ultrasound examinations, may also be used. It can be difficult with these methods to distinguish between atherosclerosis and Takayasu arteritis, particularly in older people whose tests do not show signs of inflammation.

In certain Swedish university hospitals it is also possible to use positron emission tomography (PET) to confirm inflammation of the walls of the blood vessels. This is a very sensitive technique using radioactive material.

Treatment/interventions

There is no cure for Takayasu arteritis but symptoms can be eased and it is usually possible to control the disease by medication. Many people require long-term treatment to ensure that inflammation of the blood vessels does not recur.

Cortisone is used to treat active inflammation. Treatment starts with a high dose which is gradually reduced to a lower, regular dose. It is often difficult to determine whether the individual can stop using cortisone entirely, or whether a lower dose should be maintained. Other medications may be tried in order to reduce reliance on cortisone. People who are treated with cortisone over longer periods of time should receive medication to prevent them developing brittle bones.

When the disease does not respond readily to treatment, additional immunosuppressants such as azathioprine or methotrexate are the most widely used alternatives. When other medication proves unsuccessful, cyclophosphamide, mycophenolate, IL-6 blockade and TNF blockade work well. However, as yet their effects have been insufficiently examined.

A low dose of aspirin (acetylsalicylic acid) should also be given to reduce the risk of blood clots in the damaged walls of the blood vessels. For the same reason, the person with the disease should not smoke, and high blood levels of cholesterol should be treated as in other types of heart disease. Ultrasound and MRI (magnetic resonance imaging) scans can measure the thickness of blood vessel walls very precisely, so making it possible to monitor how the walls of the blood vessels are affected.

If vital organs such as the brain and heart risk damage, medical treatment may be supplemented by heart surgery or a balloon angioplasty to widen blood vessels. These procedures should be considered only when the benefits outweigh the risks. As it can take a long time for the artery to become constricted, the body often develops new ways for the blood to circulate. There is a risk that new constrictions develop after a surgical intervention, especially if it is carried out during a bout of inflammation. Severely leaking heart valves may also require surgery.

Long-lasting symptoms following a heart attack or stroke are not affected by anti-inflammatory medication. When circulatory problems result in permanent damage to the brain or heart, rehabilitation is the same as for patients who have had strokes or heart attacks resulting from other conditions.

High dosages of cortisone may have certain side effects including abdominal fat, a swollen face, thin, fragile skin, sleep difficulties and mood changes. After the first months' treatment dosages can often be lowered to reduce side effects.

Psychological and social support may be necessary.

Practical advice

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National and regional resources in Sweden

There are resources for treating Takayasu arteritis at county and regional hospitals.

Resource personnel

Specialist Physician Mats Dehlin, Rheumatology Department, Sahlgrenska University Hospital, SE-413 45 Gothenburg, Sweden. Tel: +46 31 342 10 00, email: mats.dehlin@vgregion.se.

Senior Physician Per Eriksson, Rheumatology Department, University Hospital, SE-581 85 Linköping, Sweden. Tel: +46 10 103 00 00, email: per.eriksson@lio.se.

Courses, exchanges of experience, recreation

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Organizations for the disabled/patient associations etc.

The Swedish Rheumatism Association, Alströmergatan 39, Box 12851, SE-112 98 Stockholm, Sweden. Tel: +46 8 505 805 00, fax: +46 8 505 805 50, email: info@reumatikerforbundet.org, www.reumatikerforbundet.org.

Courses, exchanges of experience for personnel

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Research and development

General research into inflammation is under way in many places in the world, which is also important for Takayasu arteritis. Research into a closely related disease, giant cell arteritis, is being carried out in Barcelona, Spain, by Maria C. Cid, email: mccid@clinic.ub.es. Research into medical imaging technology (CT and MRI scans, ultrasound, and positron emission tomography - PET) of inflamed and non-inflamed blood vessels is being carried out by researchers including Wolfgang A. Schmidt, Medical Centre for Rheumatology Berlin-Buch, Berlin, Germany. Email: w.schmidt@immanuel.de.

Information material

Short summaries of all the database texts are available as leaflets, in Swedish only. They can be printed out or ordered by selecting the Swedish version, and then clicking on the leaflet icon which will appear under, "Mer hos oss" in the column on the right-hand side.

Literature

Bicakcigil M, Aksu K, Kenan K, Sevil A, Servet K, Omer O et al. Long-term outcome in Takayasu’s arteritis: vascular procedures performed in active or untreated patients have a poor outcome. APMIS 2009; 117 (suppl 127): 85.

Cid MC, Prieto-González S, Arguis P, Espigol-Frigolé G, Butjosa M, Hernández-Rodriguez J et al. The spectrum of vascular involvement in giant-cell arteritis: clinical consequences of detrimental vascular remodelling at different sites. APMIS 2009; 117 (suppl 127): 10-20.

Langford CA, Monach PA, Specks U, Seo P, Cuthbertson D, McAlear CA et al. Vasculitis Clinical Research Consortium. An open-label trial of abatacept (CTLA4-IG) in non-severe relapsing granulomatosis with polyangiitis (Wegener's). Ann Rheum Dis 2014; 73: 1376-1379.

Lee BB, Laredo J, Neville R, Leonel Villavicencio J. Endovascular management of Takayasu arteritis: is it a durable option? Vascular 2009; 17:1 38-146.

Maksimowicz-McKinnon K, Clark T, Hoffman G. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum 2007; 56: 1000-1009.

Molloy ES, Langford CA, Clark TM, Gota CE, Hoffman G. Anti-tumor necrosis factor therapy in patients with refractory Takayasu arteritis: long-term follow-up. Ann Rheum Dis 2008; 67: 1567-1569.

Park M-C, Lee S-W, Park Y-B, Chung NS, Lee S-K. Clinical characteristics and outcomes of Takayasu’s arteritis: analysis of 108 patients using standardized criteria for diagnosis, activity assessment, and angiographic classification. Scand J Rheum 2005; 34: 284-292.

Schmidt WA, Gromnica-Ihle E. What is the best approach to diagnosing large-vessel vasculitis? Best Pract Res Clin Reumatol 2005; 19: 223-242.

Stone JH. Vasculitis: a collection of pearls and myths. Rheum Dis Clin North Am 2007; 33: 727.

Tyagi S, Gupta MD, Singh P, Shrimal D, Girish MP. Percutaneous revascularisation of sole arch artery for severe cerebral ischemia resulting from Takayasus arteritis. J Vasc Interv Radiol 2008; 19: 1699-1703.

Takayasu M. A case with peculiar changes of the central retinal vessels. Acta Societatis ophthalmologicae Japonicae, Tokyo 1908; 12: 554.

Unizony S, Arias-Urdaneta L, Miloslavsky E, Arvikar S, Khosroshahi A, Keroack B et al. Arthritis Care Res (Hoboken) 2012; 64: 1720-1729.

Watts R, Al-Taiar A, Mooney J, Scott D, Macgregor A. The epidemiology of Takayasu arteritis in the UK. Rheumatology 2009; 48; 1008-1011.

Youngstein T, Mason JC. Interleukin 6 targeting in refractory Takayasu arteritis: Serial noninvasive imaging is mandatory to monitor efficacy. J Rheumatol 2013; 40: 1941-1944.

Database references

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Document information

The Swedish Information Centre for Rare Diseases produced and edited this information material.

The medical expert who wrote the draft of this information material is Senior Physician Per Eriksson, Linköping University Hospital, Sweden.

The relevant organizations for the disabled/patient associations have been given the opportunity to comment on the content of the text.

An expert group on rare diseases, affiliated with the University of Gothenburg, approved the material prior to publication.

Date of publication: 2014-10-16
Version: 2.0
Publication date of the Swedish version: 2014-06-23

For enquiries contact The Swedish Information Centre for Rare Diseases, The Sahlgrenska Academy at the University of Gothenburg, Box 422, SE-405 30 Gothenburg, Sweden. Tel: +46 31 786 55 90, email: ovanligadiagnoser@gu.se.

 

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This knowledge database provides information on rare diseases and conditions. The information is not intended to be a substitute for professional medical care, nor is it intended to be used as a basis for diagnosis or treatment.