--- title: Non-specific aortoarteritis (Takayasu arteritis) description: What non-specific aortoarteritis (Takayasu arteritis) is, why it develops, how it is recognised, which treatment approaches clinical guidelines describe and when to call an ambulance. type: disease aliases: - Takayasu arteritis - Takayasu disease - pulseless disease - aortic arch syndrome sources: - tier: guideline org: minzdrav-rf title: Неспецифический аортоартериит year: 2025 url: https://cr.minzdrav.gov.ru/view-cr/479_2 - tier: guideline org: aha-acc title: '2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines' year: 2022 doi: 10.1161/CIR.0000000000001106 url: https://doi.org/10.1161/CIR.0000000000001106 - tier: reference org: msd-manuals title: Takayasu Arteritis (MSD Manual Professional Version) year: 2025 url: https://www.msdmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/takayasu-arteritis sources_checked: 2026-10-13 review: null --- Non-specific aortoarteritis, or Takayasu arteritis, is inflammation of the wall of the aorta, its branches and the pulmonary arteries. The inflamed wall thickens, and the lumen of the artery narrows, sometimes until it is completely blocked; less often the wall widens and forms an aneurysm. Together with [giant cell arteritis](../giant-cell-arteritis/), this disease is classed as a large vessel vasculitis; these are the most common causes of inflammation of the aorta (aortitis). The disease is rare: it is most common in Asia but occurs worldwide, and it mainly affects young women. The Russian Ministry of Health guideline on this disease is written for children, so the figures taken from it in this article refer to children.

The diagram is a front view, so the right side of the body is on the left of the picture. Three large branches arise from the aortic arch: the brachiocephalic trunk, the left common carotid artery and the left subclavian artery. Normally the origins of the branches are not narrowed, and blood flows freely through them to the head and to both arms.

In Takayasu arteritis the inflamed wall of the aorta and its branches thickens, and the origins of the branches arising from the arch may narrow or close completely. As an example, the diagram shows one of the possible patterns: the origin of the brachiocephalic trunk is narrowed and the origin of the left subclavian artery is closed. When the arteries leading to the arms are narrowed, less blood reaches the arms: the pulse in them is weak or cannot be felt, and the blood pressure differs between the right and left arm.

## Why it happens The exact cause is unknown. It is thought that the immune system mistakenly attacks the body's own tissues, and inflammation develops in the artery wall. There is evidence of an inherited predisposition. The inflammation makes the artery wall thick, and the lumen narrows, sometimes until it is completely blocked. If the aortic arch is affected, the origins (the places where the large arteries branch off it) may become markedly narrowed or close completely. Where the wall is destroyed, the artery may widen and form an aneurysm. The parts most often affected are the ascending aorta and the aortic arch, the brachiocephalic trunk and subclavian arteries, and the common carotid and renal arteries; in about half of people, the pulmonary arteries are affected too. The disease is chronic, so bypass vessels (collaterals) have time to develop around the narrowings. Because of this, ulcers and gangrene of the arms or legs are rare. Takayasu arteritis is characterised by non-specific productive inflammation of the walls of the aorta and its branches with obliteration of their origins, and of large muscular arteries. The aetiology is unknown and probably multifactorial; there is evidence of a genetic predisposition. Cell-mediated immune mechanisms may be involved. Early changes consist of an adventitial mononuclear infiltrate with perivascular cuffing of the vasa vasorum; later, intense mononuclear inflammation of the media may occur, sometimes with granulomatous changes, giant cells and patchy necrosis. Panarteritic inflammatory infiltrates cause marked thickening of the wall and subsequent luminal narrowing and occlusion. Destruction of the elastic framework can lead to aneurysm formation. Most patients have stenoses or occlusions; aneurysms occur in approximately one-third. By the site of involvement, 5 types are distinguished (Takayasu Conference, 1994): I, the aortic arch and the arteries arising from it; IIa, the ascending aorta, the aortic arch and its branches; IIb, the same plus the descending thoracic aorta; III, the descending thoracic and abdominal aorta and/or the renal arteries; IV, the abdominal aorta and/or the renal arteries; V, a mixed form of types IIb and IV. Epidemiological data differ between the sources. MSD: a female:male ratio of 8:1, onset typically at 15–30 years, and an annual incidence in North America of about 2.6 cases per million. The paediatric Russian Ministry of Health guideline: in children, the first symptoms most often appear at 8–12 years, and among patients older than 7 years girls outnumber boys 7.5–9 to 1. ## Symptoms The disease may begin with general signs of inflammation: fever, weakness, weight loss, night sweats, and joint and muscle pain. In many people, the symptoms depend on which organ or limb is getting too little blood: - the arms hurt and tire quickly with repeated movements or when held up for a long time; the legs may hurt when walking (intermittent claudication); - the pulse in the arms or legs is weak, cannot be felt or differs between the two sides; blood pressure in one or both arms is often low; - when the arteries carrying blood to the brain are narrowed, there may be dizziness, fainting, headaches, brief problems with vision, transient ischaemic attacks and strokes; - when the origins of the coronary arteries are narrowed, angina or a heart attack (myocardial infarction); when the ascending aorta is markedly widened, aortic regurgitation; heart failure can develop; - when the renal arteries or the abdominal aorta are narrowed, blood pressure may rise; according to the Russian Ministry of Health guideline, in children this is a very common first sign of the disease; - when the pulmonary arteries are affected, pulmonary hypertension sometimes develops; when the arteries of the intestine are affected, abdominal pain and diarrhoea. Two phases of the disease are distinguished. In the acute (systemic, prestenotic) phase, non-specific signs of systemic inflammation predominate; it lasts from several weeks to several months. In the chronic (stenotic) phase, signs of vascular occlusion and ischaemia appear. Frequency of constitutional symptoms: approximately 50% of patients according to MSD; according to the paediatric Russian Ministry of Health guideline, systemic manifestations (weight loss, fever, weakness) develop in 26–86%. On examination: asymmetric or absent pulses and blood pressure; bruits over the subclavian arteries (in the supraclavicular fossa), brachial, carotid and femoral arteries and the abdominal aorta. Stenotic lesions in a subclavian artery near the origin of a patent vertebral artery can cause subclavian steal syndrome: posterior circulation ischaemic neurological symptoms or syncope when the arm is used. Obstruction of the descending thoracic aorta sometimes causes signs of aortic coarctation (hypertension, headache, leg claudication). According to the paediatric guideline, arterial hypertension in Takayasu arteritis is renovascular in origin and may be the only symptom of the disease, combined with vascular bruits. ## How it is diagnosed A doctor will suspect the disease if a person at low risk of atherosclerosis, especially a young woman, has signs of too little blood reaching the organs supplied by the aorta and its branches, a weak or absent pulse, bruits over the arteries, or a clear difference in pulse or blood pressure between the right and left arm or between an arm and a leg. That is why the pulse is checked on both sides (in the neck, at the wrists, in the groin, behind the knees and on the feet) and blood pressure is measured on both arms and both legs. Blood tests often show inflammation (raised ESR and C-reactive protein), but they cannot confirm the diagnosis; in active disease they can also be normal. The diagnosis is confirmed by imaging the whole aorta and its branches: MR angiography or CT angiography, sometimes together with positron emission tomography (PET). These scans show narrowings, blockages, irregular lumens, aneurysms and bypass vessels. Young people are more often given MRI: it involves no radiation, and the arteries will need checking for life. Echocardiography helps assess the heart and the aortic valve. The doctor rules out diseases that can look like Takayasu arteritis: for example, inherited connective tissue disorders such as [Marfan syndrome](../../disease/marfan-syndrome/), infections of the arteries (tuberculosis, syphilis) and fibromuscular dysplasia. According to the Russian Ministry of Health guideline, children with suspected disease are examined in a hospital rheumatology unit. ACC/AHA: in patients with large vessel vasculitis, prompt evaluation of the entire aorta and branch vessels with MRI or CT, with or without 18F-FDG PET, is recommended (class 1, C-LD). Imaging should be performed as soon as possible so that initiation of treatment is not delayed; the sensitivity of diagnostic imaging decreases with the duration of glucocorticoid treatment. In CT, wall thickening from inflammation may be mistaken for atherosclerosis. If proximal aortic involvement is confirmed by CT or MRI, echocardiography may be helpful to assess aortic valve function. According to the paediatric Russian Ministry of Health guideline, MR angiography is preferred in children, with CT angiography when it is not available; PET/CT is recommended when Takayasu arteritis is suspected but MR and CT angiography show no changes. Diagnostic criteria in the ACC/AHA table: age at onset under 40 years, intermittent claudication, diminished brachial artery pulse, subclavian artery or aortic bruit, systolic BP variation of more than 10 mm Hg between arms, and aortographic evidence of aorta or aortic branch stenosis; the diagnosis is established when 3 or more criteria are present (sensitivity 90.5%, specificity 97.8%). The paediatric EULAR/PRINTO/PReS 2010 criteria in the Russian Ministry of Health guideline: angiographic evidence of involvement of the aorta, its main branches or the pulmonary arteries (with other causes excluded) plus at least 1 of 5 features: asymmetric or absent pulses, a difference in systolic BP between the right and left limb of more than 10 mm Hg, loud vascular bruits, persistent arterial hypertension, persistently raised ESR or CRP. Laboratory findings are non-specific: anaemia of chronic disease, raised platelet count, occasionally raised white cell count, and raised ESR and CRP. The differential diagnosis includes inherited non-inflammatory connective tissue disorders (Ehlers–Danlos and Marfan syndromes), vascular infections (tuberculous, fungal, syphilitic), fibromuscular dysplasia, disorders causing arterial thrombosis and idiopathic inflammatory conditions (ankylosing spondylitis with aortitis, rheumatoid arthritis, Cogan and Behçet syndromes, Kawasaki disease, sarcoidosis). ## How it is treated Treatment is based on medicines that suppress inflammation: glucocorticoids (steroid hormones) together with another medicine that suppresses the immune system. The doctor chooses the medicines, doses and duration of treatment. According to the Russian Ministry of Health guideline, children with highly active disease, or whose disease stays active despite treatment, are given biological medicines. The steroid dose is lowered gradually and with frequent check-ups; stopping treatment without a doctor's supervision is dangerous. High blood pressure needs to be treated persistently. Blood pressure is measured regularly on an arm or leg whose arteries are not affected. If medicines do not help and an organ or limb is not getting enough blood, a procedure on the arteries may be needed, most often a bypass that creates a detour for the blood. According to the American guideline, it is reasonable to carry out planned procedures when the disease is quiet (in remission): the risk is lower then. The doctors decide whether a procedure is needed and which one. The course of the disease varies: in about 20% of people it occurs as a single episode; in the rest, flare-ups alternate with quiet periods or the disease gradually progresses. New narrowings can appear even when the person feels well and tests are normal, so the aorta and large arteries are checked regularly. According to the paediatric Russian Ministry of Health guideline, in children with proper treatment remission often comes within the first year. ACC/AHA: in patients with active Takayasu arteritis, initial medical therapy should include high-dose glucocorticoids (class 1, B-NR); in all patients with Takayasu arteritis, non-biological disease-modifying anti-rheumatic drugs (DMARDs) should be given in combination with glucocorticoids (class 1, C-LD), given relapse rates of up to 70%. In active disease, treatment efficacy should be periodically assessed by CRP and ESR, CT, MRI or FDG-PET imaging, and clinical symptoms (class 1, C-LD); in remission with aortic involvement, annual surveillance imaging is reasonable (class 2a, C-EO). ACC/AHA recommends an individualised approach to antithrombotic therapy, based on imaging and clinical findings of complications. The paediatric Russian Ministry of Health guideline names glucocorticoids as the main treatment for Takayasu arteritis in children. Glucocorticoids, immunosuppressants and biological agents are not recommended before a full work-up is complete and oncological, haemato-oncological and infectious diseases have been excluded. According to MSD, the optimal dose of glucocorticoids, tapering schedule and length of treatment have not been determined. Accurate measurement of BP can be difficult: if both subclavian arteries are severely affected, systemic BP can be accurately measured only in the legs, and with more extensive involvement only as central arterial pressure during angiography; otherwise occult hypertension may be missed. ACC/AHA: in remission, elective endovascular or open surgical intervention to treat aortic and branch vessel complications is reasonable (class 2a, C-LD); the decision is made by a multidisciplinary team. Indications for intervention according to MSD: aortic insufficiency, symptomatic coronary artery stenosis, dissection of an enlarged aortic aneurysm, severe renovascular hypertension refractory to medical management, limb ischaemia that interferes with daily activities, brain ischaemia, coarctation of the aorta, and inability to measure blood pressure accurately in any limb. Positions on stenting differ: according to MSD, vascular stenting is usually not recommended because the restenosis rate is high, and bypass grafting with an autologous graft has the best patency rates; the paediatric Russian Ministry of Health guideline includes stenting, endarterectomy, aneurysmectomy and balloon angioplasty of the aorta among the interventions for significant stenosis, aneurysm, difficult-to-control hypertension, angina and intermittent claudication. Aortic regurgitation may require valve surgery with aortic root replacement. A progressive course and complications (hypertension, aortic regurgitation, heart failure, aneurysms) predict a less favourable prognosis. ## When to call an ambulance Narrowing of the arteries that carry blood to the brain can cause a transient ischaemic attack or a stroke, and narrowing of the origins of the coronary arteries can cause angina or a heart attack. Possible complications affecting the aorta include aneurysms, dissection and rupture of the aorta. If there are signs of a stroke (sudden weakness or numbness on one side of the body, difficulty speaking, a drooping face), call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK) straight away. The signs of a heart attack are described in the article on [coronary heart disease](../../disease/coronary-heart-disease/), and the signs of dissection in the article on [aortic dissection](../../disease/aortic-dissection/). As with any heart condition, call an ambulance for fainting, chest pain or severe shortness of breath: this is the site's general safety rule, repeated at the bottom of every page. New general symptoms (fever, weakness, weight loss, night sweats), new pain in an arm or leg on exertion, or changes in blood pressure can be signs of disease activity: tell your doctor about them without delay. According to the Russian Ministry of Health guideline, a child with an established diagnosis who has a flare-up or complications of the disease is admitted urgently to a hospital rheumatology unit.