Definition and epidemiology
Takayasu arteritis (TA) is a chronic granulomatous large-vessel vasculitis of the aorta and its major branches, leading to vessel-wall thickening, stenosis, occlusion, aneurysm, and end-organ ischaemia. It is the third commonest vasculitis of childhood (after IgA vasculitis and Kawasaki disease) and shows a strong female predominance that increases with age. Incidence is highest in Asia, the Middle East, and Latin America. The disease is insidious: an early inflammatory (“pre-pulseless”) phase often precedes the classic occlusive (“pulseless”) phase, so diagnosis is frequently delayed.
Clinical features
The presentation reflects the combination of systemic inflammation and regional ischaemia:
- Constitutional — fever, weight loss, fatigue, night sweats, elevated acute-phase reactants.
- Vascular/ischaemic — absent or asymmetric pulses, limb claudication, inter-limb blood pressure differential, bruits over large arteries, carotidynia.
- Hypertension — very common in children (renal artery stenosis a key driver) and a major cause of morbidity.
- Cardiac/cerebral — heart failure, aortic regurgitation, headache, dizziness, syncope, visual disturbance, stroke.
Hypertension and headache are disproportionately frequent presenting features in the pediatric population compared with adults.
Diagnosis
Diagnosis is anchored on imaging. Under the validated EULAR/PRINTO/PRES (Ankara 2008) criteria, the mandatory item is angiographic abnormality (conventional, CT, or MR) of the aorta or its main branches and pulmonary arteries — aneurysm/dilatation, narrowing, occlusion, or thickened arterial wall not due to another cause — plus at least one of: pulse deficit/claudication, inter-limb BP differential >10 mmHg, bruits, hypertension, or raised acute-phase reactants (ESR/CRP). These criteria perform at high sensitivity and specificity. MR angiography is preferred in children to avoid repeated ionising radiation and characterises both lumen and wall; PET can demonstrate active wall inflammation. ESR/CRP are useful but imperfect markers of activity.
See the full criteria: Pediatric-Onset Takayasu Arteritis
Red flags
- Severe or accelerated hypertension, especially with a renal-artery bruit
- Stroke, TIA, syncope, or visual loss — critical cerebrovascular stenosis
- New aortic regurgitation or heart failure
- Rapidly worsening claudication or critical limb ischaemia
- Aortic aneurysm with dissection/rupture risk
Management overview
The goal is to suppress inflammation and halt vascular damage while managing the haemodynamic consequences (drug classes/strategy only):
- Remission induction — high-dose corticosteroids combined early with a conventional immunosuppressant (e.g. methotrexate, azathioprine, or mycophenolate; cyclophosphamide reserved for severe/refractory disease).
- Refractory disease — biologic therapy, principally anti-TNF agents or IL-6 receptor blockade, is increasingly used to achieve and maintain remission and to spare steroids.
- Adjunctive — aggressive antihypertensive management, antiplatelet therapy where indicated, and cardiovascular risk reduction.
- Vascular interventions — angioplasty/stenting or surgical bypass for critical fixed stenoses, ideally performed during quiescent (non-inflammatory) disease.
Management is multidisciplinary (pediatric rheumatology, nephrology, cardiology, vascular surgery, interventional radiology) with longitudinal imaging surveillance, since clinical and laboratory activity correlate imperfectly with progressive vascular damage.
References
- Ozen S, et al. EULAR/PRINTO/PRES Ankara 2008 criteria, Part II. Ann Rheum Dis. 2010;69:798-806.
- de Graeff N, et al. SHARE recommendations for rare paediatric vasculitides. Rheumatology. 2019;58:656-671.
- Nelson Textbook of Pediatrics, 21st ed.
Decision support for qualified clinicians only — verify against current primary guidelines and your clinical judgement.