Takayasu Arteritis
Large-vessel vasculitis predominantly affecting the aorta and its major branches, occurring primarily in young women under 40 years. Causes arterial stenosis, occlusion, and aneurysm formation leading to limb claudication, absent pulses, and renovascular hypertension.
Key Facts
Takayasu arteritis is a large-vessel vasculitis affecting the aorta and its major branches in predominantly young women (<40 years) Also called pulseless disease: absent/reduced peripheral pulses, blood pressure discrepancies between arms (>10mmHg) Most common in East Asian, South Asian, and Central/South American populations; rare in the UK Presentation: limb claudication, absent pulses, hypertension (renovascular), aortic regurgitation, bruits CT/MR angiography: wall thickening, stenoses, occlusions, aneurysms; PET-CT: active vessel wall inflammation Treatment: prednisolone 1mg/kg ± methotrexate or mycophenolate; tocilizumab or anti-TNF for refractory disease Surgical/endovascular intervention for critical stenoses: bypass grafting, angioplasty/stenting (ideally when disease is quiescent)
Overview
Key Facts
Takayasu arteritis should be considered in any young woman with unexplained hypertension, absent pulses, or limb claudication.
Epidemiology
- Incidence: 1-3 per million/year globally; higher in East Asia
- F:M 8-9:1; peak onset 15-30 years
- Very rare in Caucasian populations
Pathophysiology
- Granulomatous panarteritis of the aorta and major branches
- Intimal hyperplasia → stenosis → ischaemia
- Medial destruction → aneurysm formation
- CD4+ T-cell and macrophage-driven inflammation
- Vasa vasorum involvement → wall ischaemia
Clinical Presentation
Systemic (Early Phase)
- Fever, malaise, weight loss, arthralgia, myalgia
- Often misdiagnosed initially as infection
Vascular (Late/Occlusive Phase)
- Absent/reduced pulses in upper extremities (subclavian/axillary stenosis)
- Blood pressure discrepancy >10mmHg between arms
- Limb claudication: upper or lower limb
- Bruits: carotid, subclavian, renal, abdominal
- Renovascular hypertension: renal artery stenosis
- Aortic regurgitation: aortic root dilation
- Cerebrovascular: TIA, stroke (carotid involvement)
- Visual disturbance: retinal ischaemia
Classification (Numano)
- Type I: aortic arch branches only
- Type IIa: ascending aorta/arch
- Type IIb: ascending aorta, arch, thoracic descending
- Type III: thoracic descending, abdominal aorta, renal
- Type IV: abdominal aorta and/or renal
- Type V: entire aorta (most common overall)
Red Flags
- Young woman with unexplained hypertension → check BP in both arms
- Unrecordable BP in one arm → subclavian stenosis
- Stroke in young patient → consider Takayasu
Differential Diagnosis
| Diagnosis | Key Features | Investigation |
|---|---|---|
| GCA | Age >50, temporal artery, cranial symptoms | Temporal artery USS/biopsy |
| Atherosclerosis | Older patients, risk factors, calcification | CT angiography |
| Coarctation of aorta | Congenital, radio-femoral delay | CT aortography |
| Fibromuscular dysplasia | String-of-beads, young women | MR angiography |
| Aortitis (syphilitic, IgG4) | Specific features | Syphilis serology, IgG4 |
Diagnosis / Investigation
Bloods
- ESR/CRP: elevated in active disease (may be normal in quiescent)
- FBC: anaemia of chronic disease
Imaging (Key to Diagnosis)
- CT angiography: wall thickening, stenoses, occlusions, aneurysms; excellent for anatomy
- MR angiography: avoids radiation; good for follow-up; wall oedema on T2
- PET-CT (18F-FDG): detects active vessel wall inflammation (useful for monitoring response)
- Conventional angiography: gold standard for anatomy but invasive
- USS: carotid/subclavian artery wall thickening
Clinical
- Four-limb blood pressures: discrepancy
- Pulse assessment: all peripheral pulses
- Echocardiogram: aortic regurgitation
Management
Medical
- Prednisolone 1mg/kg/day: first-line; taper guided by symptoms and CRP/ESR/imaging
- Methotrexate 15-25mg weekly or azathioprine 2mg/kg/day or mycophenolate 2-3g/day: steroid-sparing
- Tocilizumab: effective for refractory disease (off-label but increasing evidence)
- Anti-TNF (infliximab): for refractory cases
- Cyclophosphamide: severe/life-threatening disease
Surgical/Endovascular (When Disease Quiescent)
- Bypass grafting: for critical stenoses (preferred over endovascular in many situations)
- Angioplasty/stenting: selected cases; higher restenosis rate
- Aortic valve replacement: for severe aortic regurgitation
- Timing: ideally during disease remission (surgery during active inflammation has higher complication rates)
Supportive
- Antihypertensives for renovascular hypertension
- Antiplatelet therapy: aspirin 75mg
- Exercise and rehabilitation
Referral
- All suspected Takayasu → rheumatology
- Vascular complications → vascular surgery
- Renovascular hypertension → nephrology
Prognosis
- 10-year survival: 80-90% with treatment
- Relapse rate: 50-70% during steroid taper
- Major morbidity: stroke, limb ischaemia, renal failure, aortic regurgitation
- Pregnancy: high-risk; risk of flare, hypertension, pre-eclampsia
- Earlier diagnosis and treatment associated with better outcomes
- Many patients require lifelong immunosuppression
Other Relevant Information
Takayasu vs GCA
| Feature | Takayasu | GCA |
|---|---|---|
| Age | <40 | >50 |
| Sex | F >>> M | F > M |
| Vessels | Aorta + branches | Temporal + aorta |
| Ethnicity | Asian, South American | Northern European |
| Pathology | Granulomatous (identical to GCA) | Granulomatous |
| Treatment | Steroids ± DMARD | Steroids ± tocilizumab |