📋 Key Information Summary
- Takayasu arteritis (TAK) is a chronic granulomatous large-vessel vasculitis predominantly affecting the aorta and its primary branches, causing progressive stenosis, occlusion, or aneurysm formation.
- Young women are predominantly affected — female-to-male ratio approximately 8:1, with peak onset between 15 and 30 years of age.
- Also known as "pulseless disease" — characteristic absent or diminished peripheral pulses, limb claudication, and blood pressure discrepancy between arms (>10 mmHg systolic difference).
- HLA-B52 association is well documented, particularly in Asian populations; also associated with increased disease severity and aortic regurgitation.
- ACR/EULAR 2022 classification criteria use a weighted scoring system (≥5 points = classified as TAK): age at onset ≤40 years, female sex, angina, limb claudication, arterial bruit, reduced pulse, BP discrepancy, and imaging findings.
- Numano angiographic classification (types I–VI) describes the pattern of vessel involvement and guides treatment planning and prognosis.
- Diagnosis requires cross-sectional imaging — CTA or MRA of the entire aorta showing concentric vessel wall thickening; FDG-PET may demonstrate active vascular inflammation before luminal changes appear.
- First-line therapy is high-dose corticosteroids — prednisolone 1 mg/kg/day (max 60 mg), with a slow taper over 6–12 months; relapse rate is high (60–80%) with steroids alone.
- Steroid-sparing agents are almost universally required — methotrexate (first-line conventional DMARD), azathioprine, or mycophenolate mofetil.
- Biologic agents for refractory disease — anti-TNF agents (infliximab, adalimumab) or tocilizumab (anti-IL-6R) are recommended when conventional DMARDs fail; both are PBS Authority Required in Australia.
- Revascularisation procedures (surgical bypass or endovascular angioplasty/stenting) should only be performed during stable clinical remission, ideally ≥6 months of quiescent disease on imaging.
- Indefinite immunosuppression is generally recommended given the high relapse risk on withdrawal; long-term cardiovascular risk management is essential.
- Aboriginal and Torres Strait Islander Australians may present with more advanced vascular damage at diagnosis; culturally safe, multidisciplinary care and remote access pathways are essential.
Introduction & Australian Epidemiology
Takayasu arteritis (TAK) is a chronic, granulomatous large-vessel vasculitis that predominantly affects the aorta and its primary branches. Progressive inflammation of the vessel wall leads to stenosis, occlusion, and — less commonly — aneurysm formation. The disease was first described by Mikito Takayasu in 1908, who noted retinal arteriovenous anastomoses in a young woman with absent radial pulses.
The clinical course typically progresses through three overlapping phases: (1) a systemic inflammatory (pre-pulseless) phase with constitutional symptoms such as fever, malaise, arthralgia, and weight loss; (2) a vascular inflammatory phase with arteritis causing vessel wall thickening and pain; and (3) a "burnt-out" or fibrotic phase characterised by fixed stenotic and occlusive lesions leading to ischaemic complications including limb claudication, renovascular hypertension, and cerebrovascular insufficiency.
Australian context: TAK is rare in Australia, with an estimated prevalence of 2–3 per million. However, it is the most common childhood-onset large-vessel vasculitis and carries significant morbidity if not diagnosed early. Australian tertiary vasculitis centres (e.g., Royal Adelaide Hospital, Monash Health, St Vincent's Hospital Melbourne) manage the majority of cases. The disease may be under-recognised in remote and Indigenous communities where access to specialist imaging and rheumatology services is limited.
Diagnostic delay is common: The median delay from symptom onset to diagnosis in TAK is 10–15 months. The systemic phase is often misdiagnosed as infection, autoimmune disease, or psychosomatic illness. Maintain a high index of suspicion in any young patient (especially women <40 years) presenting with unexplained limb claudication, blood pressure discrepancy, new-onset renovascular hypertension, or constitutional symptoms with raised inflammatory markers.
Epidemiology & Classification
Epidemiology
TAK is rare in Western populations (incidence 1–3 per million per year) but more common in Asia, particularly Japan, India, China, and Southeast Asia, where incidence may reach 15–40 per million per year. It predominantly affects women (female-to-male ratio approximately 8:1) with a peak age of onset between 15 and 30 years. Disease onset after age 40 is uncommon and should prompt consideration of giant cell arteritis (GCA), particularly in patients of Northern European descent.
| Feature | Detail |
|---|---|
| Global prevalence | 4.7–26 per million (higher in Asia) |
| Australian prevalence | Estimated 2–3 per million |
| Female : Male ratio | 8 : 1 |
| Peak onset age | 15–30 years |
| Ethnic predominance | Asian > Hispanic > African > Caucasian |
| HLA association | HLA-B52 (especially Japanese, Korean populations) |
| 5-year survival (untreated) | ~80–90% |
| 10-year event-free survival | ~50–60% with treatment |
Genetic & Immunological Factors
HLA-B52 is the strongest genetic risk factor, particularly in East Asian populations, where it confers an odds ratio of approximately 3–5. HLA-B52-positive patients are more likely to have aortic regurgitation, systemic hypertension, and more extensive aortic involvement. Polymorphisms in IL-12B, FCGR2A, and TNFAIP3 have also been implicated in genome-wide association studies. The immunopathogenesis involves an initial innate immune response in the adventitia, with subsequent T-cell (particularly Th1 and Th17) mediated granulomatous inflammation propagating through the vessel wall.
ACR/EULAR 2022 Classification Criteria
The 2022 ACR/EULAR classification criteria replaced the earlier 1990 criteria and use a weighted additive scoring system. Patients with suspected vasculitis who score ≥5 points are classified as having TAK.
| Criterion | Points |
|---|---|
| Age at onset ≤40 years | +2 |
| Female sex | +1 |
| Angina or ischaemic pain | +2 |
| Limb claudication | +2 |
| Vascular bruit (subclavian/abdominal aorta) | +2 |
| Reduced/absent pulse, pulse asymmetry, or BP discrepancy ≥20 mmHg | +2 |
| Bilateral subclavian or axillary artery involvement on imaging | +3 |
| Concentric wall thickening of ≥2 large arteries on imaging | +1 |
| Involvement of ≥2 large-vessel territories | +1 |
| Saccular or fusiform aneurysm | +1 |
| Requires exclusion of other vasculitides, fibromuscular dysplasia, and IgG4-related disease | |
Numano Angiographic Classification
The Numano classification describes the anatomical pattern of vascular involvement and helps guide therapeutic and surgical planning:
| Type | Vessels Involved | Prevalence |
|---|---|---|
| Type I | Branches of the aortic arch | ~15–20% |
| Type IIa | Ascending aorta, aortic arch, and its branches | ~10–15% |
| Type IIb | Type IIa + descending thoracic aorta | ~15–20% |
| Type III | Descending thoracic aorta, abdominal aorta, and/or renal arteries | ~10–15% |
| Type IV | Abdominal aorta and/or renal arteries | ~15–20% |
| Type V | Combined features of types IIb and IV (entire aorta) | ~20–30% |
Type V disease is the most common pattern in Asian cohorts, whereas type I (branch vessel-only) disease may be more common in European series. The pattern of involvement influences complications — type IIa is associated with aortic regurgitation and coronary ostial stenosis, while types III–IV are more commonly associated with renovascular hypertension.
Pathophysiology
TAK is characterised by granulomatous inflammation of the vessel wall, primarily affecting the media and adventitia of the aorta and its major branches. The immunopathogenesis involves both innate and adaptive immune responses:
- Initiation: A triggering event (possibly infectious or mechanical stress at the aortic root) activates dendritic cells in the adventitia, producing IL-12, IL-18, and IL-6.
- Adaptive response: Activated dendritic cells promote Th1 (IFN-γ-producing) and Th17 (IL-17-producing) responses. Cytotoxic CD8+ T cells and natural killer (NK) cells contribute to vascular wall destruction.
- Vasa vasorum: Obliterative panarteritis of the vasa vasorum compromises nutrient supply to the vessel wall, accelerating tissue ischaemia and fibrosis.
- Vascular remodelling: Progressive intimal hyperplasia and adventitial fibrosis lead to luminal stenosis. Degradation of the elastic lamina may result in aneurysm formation.
- B-cell contribution: B-cell lymphoid neogenesis occurs within the vessel wall, and rituximab has shown efficacy in refractory cases, supporting a pathogenic role for B cells.
Clinical significance: The dual inflammatory and fibrotic nature of TAK means that elevated inflammatory markers (ESR, CRP) may normalise despite ongoing active wall inflammation detectable by PET or MRI. Conversely, fixed stenotic lesions from fibrosis will not improve with immunosuppression alone.
Clinical Features & Diagnostic Criteria
Three Phases of Disease
The clinical presentation of TAK progresses through overlapping phases, although not all patients experience a recognisable systemic prodrome:
Phase 1
Systemic (Inflammatory) Phase
Fever, fatigue, weight loss, arthralgia, myalgia, night sweats, headache, and general malaise. This phase is often mistaken for infection, lymphoma, or connective tissue disease. Duration: weeks to months.
Setting: GP / ED assessment
Phase 2
Vascular Inflammatory Phase
Vessel pain (carotidyngia — tenderness along the carotid arteries), new arterial bruits, limb claudication, blood pressure discrepancy between arms, and progressive pulse deficits. Carotidyngia is a highly specific symptom.
Setting: Specialist referral
Phase 3
"Burnt-Out" (Fibrotic) Phase
Fixed vascular stenoses and occlusions causing renovascular hypertension, aortic regurgitation, coronary ostial stenosis, cerebrovascular ischaemia, limb ischaemia, and aneurysm formation. Inflammatory markers may normalise.
Setting: Multidisciplinary (rheumatology, vascular surgery, interventional radiology)
Clinical Features by Vascular Territory
| Territory | Manifestation | Frequency |
|---|---|---|
| Subclavian/axillary | Upper limb claudication, absent/radial pulse, BP discrepancy >10 mmHg, Raynaud-like symptoms | 80–90% |
| Carotid/vertebral | Carotidyngia, bruits, TIA, stroke, visual disturbances, dizziness | 50–60% |
| Renal arteries | Renovascular hypertension (often severe and resistant), renal impairment | 30–40% |
| Ascending aorta/aortic root | Aortic regurgitation, aortitis, coronary ostial stenosis | 20–30% |
| Descending/abdominal aorta | Abdominal aortic aneurysm, mesenteric ischaemia, lower limb claudication | 40–60% |
| Pulmonary arteries | Pulmonary hypertension, pulmonary artery stenosis (often asymptomatic) | 15–20% |
Key Examination Findings
- Blood pressure discrepancy: Systolic BP difference ≥10 mmHg between arms — perform bilateral BP measurement in all suspected cases.
- Pulse deficits: Absent, diminished, or delayed radial, brachial, or carotid pulses.
- Arterial bruits: Listen over the carotid, subclavian, abdominal aorta, and femoral arteries.
- Carotidyngia: Palpable tenderness over the carotid arteries — a distinguishing feature from atherosclerotic disease.
- Aortic regurgitation murmur: Early diastolic murmur at the left sternal border in type IIa disease.
Red flags requiring urgent assessment: New-onset stroke or TIA in a young woman, uncontrolled renovascular hypertension, symptomatic limb ischaemia, progressive aortic regurgitation, or aortic aneurysm expansion. These patients require emergency specialist referral and may need urgent surgical intervention.
Investigations
Laboratory Investigations
MBS Available
ESR & CRP
Elevated in ~60% of active disease; normal levels do not exclude active inflammation. Monitor serially. MBS Item 65070 (ESR), 65090 (CRP).
MBS Available
Full Blood Count
Normocytic anaemia of chronic disease, thrombocytosis in active disease. MBS Item 65070.
MBS Available
Renal Function (UEC)
Baseline and to assess renovascular impairment. MBS Item 66515.
MBS Available
HLA-B52 typing
Supportive if positive (particularly in Asian populations); does not confirm or exclude diagnosis. MBS Item 71139 (HLA typing).
MBS Available
ANCA, ANA, dsDNA
To exclude ANCA-associated vasculitis and SLE. TAK is typically seronegative.
MBS Available
Lipid profile, HbA1c
Cardiovascular risk assessment — TAK patients have accelerated atherosclerosis.
Imaging
Essential
CT Angiography (CTA) — entire aorta
First-line imaging for suspected TAK. Demonstrates concentric vessel wall thickening, luminal stenosis, occlusion, and aneurysm formation. The entire aorta and branch vessels should be imaged from the aortic arch to the iliac bifurcation. MBS Item 57353 (CT aortogram). Requires contrast; caution in renal impairment.
Essential
MR Angiography (MRA) — entire aorta
Preferred in young patients to avoid ionising radiation. Can detect vessel wall oedema (bright signal on T2-weighted images) as a marker of active inflammation. MBS Item 63001 (MRI with MRA). Gadolinium contrast required for optimal assessment.
Specialist
FDG-PET/CT
Increasing role in detecting active vascular inflammation before luminal changes are evident. Useful for distinguishing active vasculitis from damage. Not universally available in Australia; tertiary centre access. May not be MBS-rebated for TAK specifically — prior approval may be required.
MBS Available
Echocardiography
Assess aortic regurgitation, aortic root dilation, and pulmonary hypertension. Annual surveillance recommended. MBS Item 55121.
MBS Available
Duplex ultrasound
Non-invasive assessment of carotid, subclavian, and limb arteries. Can detect wall thickening and stenosis. MBS Item 55700 series.
Risk Stratification & Disease Activity Assessment
Assessment of Disease Activity
Disease activity assessment in TAK is challenging because traditional inflammatory markers (ESR, CRP) may be normal despite active disease, and established vascular damage does not improve with immunosuppression. Multiple tools should be used in combination:
| Tool | Details | Limitations |
|---|---|---|
| ITAS2010 | Indian Takayasu Clinical Activity Score — 44 items; ITAS-A includes ESR. Active disease: ITAS ≥1 or ITAS-A ≥2 | May over-score irreversible damage |
| Kerr criteria | ≥2 of: systemic symptoms, elevated ESR, new/worsening claudication, new bruit, new BP discrepancy, new pulse deficit | Lower sensitivity for subclinical activity |
| DEI-TAK | Disease Extent Index for TAK — assesses extent of vascular involvement | Measures extent not activity |
| Imaging | Vessel wall oedema on MRI, FDG uptake on PET, new/worsening lesions on CTA/MRA | FDG-PET availability; radiation with serial CTA |
Factors Predicting Relapse
- Young age at onset (<20 years)
- Type V (extensive) disease
- Involvement of the ascending aorta or aortic root
- Elevated CRP at time of apparent remission
- Persistent vessel wall oedema on MRI
- Rapid corticosteroid taper
Empirical & First-Line Therapy
Induction — Corticosteroids
High-dose corticosteroids are the cornerstone of initial therapy and produce remission in approximately 50–60% of patients as monotherapy. However, relapse rates on steroid taper are high (60–80%), and steroid-sparing agents should be started early.
Prednisolone
Solone® · Panafcortelone® · Generic · Corticosteroid
Adult dose
1 mg/kg/day (max 60 mg) PO for 2–4 weeks; taper by 5 mg every 2 weeks to 20 mg/day, then by 2.5 mg every 2–4 weeks; target ≤5 mg/day by 6–12 months
Paediatric dose
1–2 mg/kg/day (max 60 mg); taper guided by paediatric rheumatology
Route
Oral (PO); IV methylprednisolone 500–1000 mg/day × 3 days for severe/rapidly progressive disease
Duration
Induction 6–12 months; low-dose maintenance (≤5 mg) may be continued indefinitely
Renal adjustment
No dose adjustment; monitor fluid retention
PBS status
✔ PBS General Benefit
Steroid side effects are significant: Bone protection (calcium, vitamin D, consider bisphosphonate if >3 months of steroids), glucose monitoring, PJP prophylaxis if high-dose steroids + immunosuppression, and cardiovascular risk factor management are all essential.
Steroid-Sparing Agents — Conventional DMARDs
Conventional DMARDs should be co-initiated with corticosteroids in virtually all patients given the high relapse rate. The choice depends on patient factors including pregnancy planning, hepatic function, and thiopurine methyltransferase (TPMT) status.
Methotrexate
Methoblastin® · Generic · DMARD (1st-line steroid-sparing)
Adult dose
15–25 mg PO/SC once weekly; folic acid 5 mg weekly (not same day)
Paediatric dose
10–15 mg/m² PO/SC once weekly (max 25 mg); folic acid 5 mg weekly
Key monitoring
FBC, LFTs, UEC every 2 weeks initially, then monthly; avoid in significant hepatic/renal disease
Contraception
MANDATORY effective contraception; teratogenic. Washout 3 months before conception (males and females)
PBS status
✔ PBS General Benefit
Azathioprine
Imuran® · Generic · DMARD (alternative 1st-line)
Adult dose
2–2.5 mg/kg/day PO; start 50 mg/day and uptitrate over 2–4 weeks
Paediatric dose
1–2 mg/kg/day PO
Key monitoring
Check TPMT/NUDT15 genotype before starting. FBC fortnightly for first 2 months, then monthly. LFTs monthly.
PBS status
✔ PBS General Benefit
Mycophenolate mofetil
CellCept® · Generic · DMARD (2nd-line conventional)
Adult dose
1–1.5 g PO BD; start 500 mg BD and uptitrate over 2–4 weeks
Key monitoring
FBC, LFTs monthly. Teratogenic — effective contraception mandatory.
PBS status
✔ PBS General Benefit (for organ transplant; TAK use may require Authority for vasculitis indication or private script)
Directed / Biologic Therapy for Refractory Disease
For patients who relapse on or fail to respond to conventional DMARDs, biologic agents targeting TNF-α or IL-6 receptor have demonstrated efficacy. There is no randomised head-to-head trial; choice is based on comorbidities, patient preference, and PBS access.
Tocilizumab
Actemra® · Anti-IL-6 receptor monoclonal antibody
Adult dose
162 mg SC weekly (preferred for TAK) or 8 mg/kg IV every 4 weeks
Efficacy
Phase III trials (TAKT, GiACTA) showed reduced relapse rates; effective in steroid-sparing. CRP may be unreliable for disease monitoring during treatment (IL-6 blockade suppresses CRP).
Key monitoring
LFTs, lipid profile (may cause hyperlipidaemia), neutrophil count. Increased infection risk. Screen for TB (QuantiFERON) before commencing.
PBS status
⚠ Authority Required — PBS authority for giant cell arteritis (may apply for TAK under rheumatology specialist). Private script otherwise ~0/month.
Infliximab
Remicade® · Inflectra® · Renflexis® · Anti-TNF-α chimeric antibody
Adult dose
5 mg/kg IV at weeks 0, 2, 6, then every 6–8 weeks
Efficacy
Open-label studies show remission rates of 60–80% in refractory TAK. Effective for patients failing MTX/AZA.
Key monitoring
TB screening (QuantiFERON/Gold) mandatory. Hepatitis B serology. Monitor for infusion reactions. Check drug/anti-drug antibody levels if loss of response.
PBS status
⚠ Authority Required — PBS for approved indications (RA, Crohn's, etc.); TAK use requires specialist authority application or private script.
Adalimumab
Humira® · Hadlima® · Anti-TNF-α fully human antibody
Adult dose
40 mg SC every 2 weeks
Key advantage
Self-administered SC injection; suitable for patients unable to attend infusion centres (e.g., remote Australia).
PBS status
⚠ Authority Required — as per infliximab; TAK is not a listed PBS indication. May require private script or authority application.
Other agents with emerging evidence: Rituximab (anti-CD20) has shown benefit in refractory cases and may be considered where TNF/IL-6 inhibition fails. Abatacept (CTLA-4 Ig) showed benefit in a randomised trial (ABATAcept for the Treatment of Relapsing/Refractory Takayasu Arteritis — ABATAS trial). JAK inhibitors (e.g., upadacitinib) are under investigation. These are not PBS-listed for TAK.
Monitoring & Follow-Up
Routine Monitoring Schedule
| Parameter | Frequency | Notes |
|---|---|---|
| ESR, CRP | Every 1–3 months (active disease); every 3–6 months (remission) | Normal values do not exclude active disease |
| FBC, LFTs, UEC | Monthly initially with DMARDs; every 2–3 months once stable | DMARD toxicity monitoring |
| Bilateral blood pressure | Every visit | Document arm used; monitor discrepancy |
| Pulse examination | Every visit | Record all palpable pulses; note new deficits |
| Vascular imaging (CTA or MRA) | Every 6–12 months (active); annually (remission) | MRA preferred to minimise radiation in young patients |
| Echocardiography | Annually | Assess aortic regurgitation, root dilation |
| FDG-PET (if available) | When clinical/imaging discordance | Detect subclinical inflammation |
| DEXA scan | Baseline and annually if on prednisolone >5 mg | Osteoporosis prevention |
| Cardiovascular risk assessment | Annually | Lipids, HbA1c, smoking status, Framingham risk |
Definition of Remission
Complete remission in TAK requires: (1) absence of systemic symptoms attributable to active vasculitis; (2) no new vascular symptoms (claudication, pulse deficits, BP discrepancy); (3) normalisation or stable/improving inflammatory markers (ESR, CRP); and (4) absence of new/worsening vascular lesions on imaging.
Clinical remission ≠ histological remission: Active inflammation may persist in the vessel wall despite normal inflammatory markers. Vessel wall oedema on MRI or FDG uptake on PET can detect subclinical activity. Treatment withdrawal should be approached cautiously — most experts recommend indefinite low-dose immunosuppression.
Surgical & Endovascular Revascularisation
Vascular intervention is indicated for critical stenoses causing symptomatic ischaemia (limb-threatening, cerebrovascular, renovascular hypertension refractory to medical therapy). Procedures should only be performed during stable clinical and serological remission — ideally ≥6 months of disease quiescence — as intervention during active inflammation carries a high risk of restenosis and graft failure.
Interventional Options
| Procedure | Indication | Considerations |
|---|---|---|
| Surgical bypass grafting | Long-segment occlusions, aortic arch syndrome, complex renovascular disease | Higher durability than endovascular; requires expertise in large-vessel surgery; use autologous vein where possible |
| Percutaneous transluminal angioplasty (PTA) | Short-segment stenoses (especially renal arteries) | Lower procedural risk; higher restenosis rate than surgery; avoid stenting in inflamed vessel wall |
| Aortic valve replacement | Severe aortic regurgitation | Mechanical vs bioprosthetic valve — use a bioprosthesis if future immunosuppression is planned |
| Endovascular aortic repair (EVAR/TEVAR) | Aortic aneurysm | May be suitable for anatomically favourable aneurysms; long-term data limited in TAK |
Do not intervene during active disease: Vascular procedures performed during active inflammation have unacceptably high rates of restenosis, anastomotic pseudoaneurysm, and graft failure. Continue immunosuppression perioperatively and ensure stable remission before proceeding.
Special Populations
Pregnancy
Prednisolone Safe in pregnancy; use lowest effective dose. Monitor for gestational diabetes.
Azathioprine Safe in pregnancy — preferred steroid-sparing agent. TPMT testing essential.
Methotrexate TERATOGENIC — contraindicated. Wash out ≥3 months before conception.
Mycophenolate TERATOGENIC — contraindicated. Wash out ≥6 weeks before conception.
Anti-TNF agents Considered low risk; infliximab/adalimumab can be continued in 1st/2nd trimester. Discontinue by 32 weeks to avoid neonatal immunosuppression.
Tocilizumab Limited data; stop ≥3 months before planned conception. Case reports suggest safety but insufficient evidence.
Management: Plan pregnancy during stable remission. Pre-conception counselling mandatory. Multidisciplinary care with obstetrics, rheumatology, and vascular surgery. Monitor BP closely — pre-eclampsia risk is elevated. Renovascular hypertension may worsen in pregnancy.
Paediatric TAK
Prednisolone 1–2 mg/kg/day; growth retardation is a major concern — use steroid-sparing agents early.
Methotrexate 10–15 mg/m²/week; commonly used as first steroid-sparing agent in children.
Biologic agents Infliximab and tocilizumab increasingly used in paediatric refractory TAK; specialist tertiary centre oversight.
Considerations: Higher relapse rates than adult-onset disease. BP monitoring in paediatric patients requires age-appropriate cuff sizes. Growth and pubertal development require close monitoring. Transition to adult services needs careful planning. MRA preferred over CTA to reduce lifetime radiation exposure.
Elderly (>50 years)
Diagnostic challenge: Onset after age 50 is atypical for TAK. Consider giant cell arteritis (GCA), which also affects large vessels and may overlap. Biopsy of temporal artery and/or FDG-PET may help differentiate.
Steroid caution: Higher risk of steroid-related complications (osteoporosis, diabetes, infections). Initiate bone protection early. Lower threshold for steroid-sparing agents.
Atherosclerosis: Distinguishing TAK from atherosclerotic disease may be challenging. Concentric wall thickening and involvement of atypical segments favour TAK.
Renal Impairment
Renovascular hypertension Common presentation (30–40% of TAK). May require ACE inhibitors cautiously if bilateral renal artery stenosis excluded. Consider PTA for refractory renovascular HTN.
Methotrexate Avoid or reduce dose if eGFR <30 mL/min. Switch to azathioprine or mycophenolate.
CTA contrast Avoid iodinated contrast if eGFR <30; use MRA (avoid gadolinium if eGFR <30 — use non-contrast MRA techniques).
Hepatic Impairment
Methotrexate Contraindicated in significant liver disease or heavy alcohol use. Monitor LFTs closely.
Azathioprine Hepatotoxicity risk; monitor LFTs. Dose reduction if significant hepatic impairment.
Consider: Mycophenolate or tocilizumab as alternatives with less hepatic toxicity.
Immunocompromised
Infections: Screen for latent TB (QuantiFERON), hepatitis B/C before biologic therapy. Ensure vaccinations are up to date (pneumococcal, influenza, COVID-19) before starting immunosuppression. Avoid live vaccines on immunosuppression.
PJP prophylaxis: Trimethoprim-sulfamethoxazole 480 mg daily (or 960 mg 3×/week) if prednisolone ≥20 mg/day for ≥4 weeks combined with another immunosuppressant.
Aboriginal and Torres Strait Islander Health Considerations
Aboriginal and Torres Strait Islander Health
While large-vessel vasculitis data specific to Aboriginal and Torres Strait Islander populations are limited, there are important considerations for equitable and culturally safe care in the Australian context.
Resources: RHDAustralia (rhdaustralia.org.au) for rheumatic disease pathways; NACCHO (naccho.org.au) for Aboriginal Community Controlled Health Organisation networks; PATS for transport assistance; Specialist Outreach services for remote rheumatology consultation.
📚 References
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