Takayasu's arteritis

Clinical manifestations according to affected areas

• Early systemic phase (inflammatory): moderate fever (38–39 °C) + profound fatigue + joint pain + muscle pain + night sweats + weight loss + headaches + elevated ESR and CRP → often diagnosed as fever of unknown origin + chronic infection + or tuberculosis (TB and tuberculosis share a geographic association + and tuberculosis can trigger TB)
• Involvement of the subclavian and axillary arteries (most frequent): Absent or asymmetrical radial pulse + upper limb claudication (pain + arm fatigue on exertion) + blood pressure difference between arms (> 10 mmHg = significant + > 20 mmHg = strong suspicion) + vascular bruits in the axillary and subclavian fossae + the classic «absent pulse» = consequence of subclavian stenoses
• Carotid and vertebral artery disease Ischemic strokes + TIAs + syncope + headaches + visual disturbance + carotid bruit + dilatation and tortuosity of retinal arteries (retinal arteriovenous anastomoses described by Takayasu)
• Aortic coarctation (acquired aortic coarctation): Upper extremity hypertension + lower extremity hypotension + high arm/ankle pressure difference + lower extremity claudication + postprandial abdominal pain (mesenteric ischemia if mesenteric arteries are affected)
• Renal artery disease Renovascular hypertension ++ (main cause of hypertension in young women in endemic countries) + Severe refractory hypertension + Renal echo-Doppler (renal artery stenosis) + Kidney failure
• Coronary involvement rare + IDM in a young woman without classic risk factors → consider TA + coronary arteritis
• Aortic insufficiency aortic root dilatation + progressive aortic regurgitation + heart failure + valvular surgery if severe

Diagnostic Criteria—ACR 1990 (6 criteria, 3 required)

• Criterion 1 — Age of onset ≤ 40 years
• Criterion 2 — Limb Claudication: fatigue or discomfort of the extremity muscles during use
• Criterion 3 - Decrease in brachial pulse: Diminished pulse in the brachial artery (one or both arms)
• Criterion 4 — Blood pressure difference between arms > 10 mmHg
• Criterion 5 — Bruit over the subclavian artery or aorta: auscultated souffle on the abdominal aorta + the subclavian artery + or the iliac arteries
• Criterion 6 — Arteriographic anomaly: narrowing + occlusion + or aneurysm of the aorta + its branches + or proximal pulmonary arteries not explained by another cause + sensitivity 90.5 % + specificity 97.8 % if ≥ 3 criteria

Vascular imaging

Treatment

• Systemic corticosteroids (first-line treatment): prednisone 40–60 mg/day → remission in 60–100 % of cases in active inflammatory phase + gradual tapering over 1–2 years + relapse in 50–70 % of cases during tapering → frequent need for prolonged maintenance therapy or corticosteroid-sparing agents
• Methotrexate (corticosteroid-sparing — 2nd line): 15–25 mg/week + folic acid + effective for maintaining remission + reduce corticosteroid dose + time to efficacy 4–8 weeks
• Mycophenolate mofetil + azathioprine: Alternatives if methotrexate is not tolerated or insufficient
• Tocilizumab (anti-IL-6R): Reference biotherapy for relapse or corticosteroid dependence + TAKT clinical trial (2017) inconclusive for relapse reduction but favorable observational data + 162 mg SC/week + or 8 mg/kg IV/4 weeks + approved in some countries for refractory RA + QuantiFERON mandatory before
• Anti-TNF (infliximab + adalimumab): positive observational data in methotrexate-refractory forms + or if tocilizumab unavailable + QuantiFERON mandatory
• Vascular treatment (endovascular or surgical): Percutaneous angioplasty ± stent (symptomatic renal + subclavian artery stenoses) + aortocoronary or aortorenal bypass + aneurysm resection + aortic replacement → ideally performed during remission (controlled inflammation) to reduce the risk of restenosis + increased perioperative mortality if performed during active phase
• Aspirin + statins: Cardiovascular risk reduction + aspirin 81–100 mg/day if no contraindications (risk of stroke + thrombosis)

Consult at Clinique Omicron

Clinique Omicron's physicians and nurse practitioners (NPs) consider Takayasu arteritis in any unexplained hypertension in young women, along with prolonged fever of unknown origin, asymmetrical pulses, or vascular bruits. They order initial investigations (ESR + CRP + CBC + creatinine + lipid profile + imaging), measure blood pressure in all four limbs, refer to rheumatology, cardiology, and vascular surgery for multidisciplinary management, and monitor cardiovascular comorbidities and the effects of immunosuppressive treatments. Consultations are available at several service points across Quebec and via telemedicine. To book an appointment, visit cliniqueomicron.ca.

The content of this page is for informational purposes only and does not replace the advice of a doctor or a rheumatologist specializing in vasculitis. Takayasu's arteritis is a rare disease that requires long-term specialized multidisciplinary follow-up. CRP and ESR may be normal despite persistent inflammatory activity — PET-CT is more sensitive for detecting subclinical vascular activity.