Cardiology >  Takayasu Arteritis  

Background

Takayasu arteritis (TA) is a chronic, large-vessel granulomatous vasculitis that primarily affects the aorta and its major branches. It leads to arterial stenosis, occlusion, or aneurysm formation. Inflammation within the vessel wall disrupts normal blood flow, potentially causing end-organ ischemia, claudication, and systemic symptoms. It predominantly affects young women and may progress silently before presenting with significant vascular compromise. 

II) Classification/Types

By Clinical Phase: 

• • Systemic Phase: Nonspecific symptoms like fever, malaise, weight loss, arthralgias 

• • Occlusive Phase: Ischemic symptoms due to vascular stenosis or aneurysm formation 

By Vascular Involvement (Numano Classification): 

• • Type I: Branches of the aortic arch 

• • Type IIa: Ascending aorta, aortic arch, and branches 

• • Type IIb: Same as IIa plus thoracic descending aorta 

• • Type III: Thoracic descending aorta, abdominal aorta, and/or renal arteries 

• • Type IV: Abdominal aorta and/or renal arteries 

• • Type V: Combined involvement of the above areas 

III) Pathophysiology 

TA is characterized by granulomatous inflammation of the tunica media and adventitia, leading to arterial wall thickening, fibrosis, stenosis, or aneurysmal dilation. Immune-mediated damage involving T cells, macrophages, and cytokines plays a central role. Chronic inflammation may eventually lead to arterial occlusion or ischemia of affected organs. 

IV) Epidemiology

• • Sex: Strong female predominance (up to 90%) 

• • Age: Commonly affects individuals <40 years 

• • Geography: More prevalent in Asia, Africa, and Latin America 

• • Comorbidities: May coexist with other autoimmune conditions (e.g., inflammatory bowel disease) 

 
Etiology

I) Causes

• • Idiopathic in most cases 

• • Potential autoimmune basis with HLA associations (e.g., HLA-B52) 

• • Possible infectious triggers (e.g., tuberculosis) 

II) Risk Factors

• • Female sex 

• • Age 10–40 years 

• • Asian ancestry 

• • Family history of autoimmune diseases 

• • Prior or concurrent tuberculosis infection 

Clinical Presentation

I) History (Symptoms)

Systemic Phase: 

• • Fatigue 

• • Low-grade fever 

• • Night sweats 

• • Weight loss 

• • Arthralgias or myalgias 

Occlusive Phase: 

• • Limb claudication (especially upper extremities) 

• • Dizziness or syncope 

• • Visual disturbances 

• • Carotidynia 

• • Hypertension (from renal artery stenosis) 

• • Chest pain (aortic involvement or coronary artery ischemia) 

II) Physical Exam (Signs)

Vital Signs: 

• • Blood pressure discrepancies between limbs 

• • Hypertension (if renal arteries involved) 

Vascular Exam: 

• • Absent or diminished pulses (“pulseless disease”) 

• • Bruits over subclavian, carotid, abdominal, or femoral arteries 

• • Asymmetric limb blood pressure 

Ocular: 

• • Retinal ischemia (e.g., visual field deficits, amaurosis fugax) 

Cardiovascular: 

• • Aortic regurgitation (if aortic root dilation) 

• • Left ventricular hypertrophy (due to systemic hypertension) 

Differential Diagnosis (DDx)

• • Giant cell arteritis (older age, cranial symptoms) 

• • Atherosclerosis 

• • Fibromuscular dysplasia 

• • Infective endocarditis (with embolic events) 

• • Systemic lupus erythematosus 

• • Polyarteritis nodosa 

• • Coarctation of the aorta (especially in young hypertensives) 

• • Rheumatoid vasculitis 

Diagnostic Tests

Initial Tests: 

• • Inflammatory Markers: 

• • • ESR and CRP typically elevated during active disease 

• • Imaging: 

• • • MRI/MRA: Preferred for detecting vessel wall edema and stenosis 

• • • CT Angiography: Detects vascular stenosis, wall thickening, and aneurysms 

• • • Conventional Angiography: Gold standard for vascular anatomy (reserved for intervention planning) 

• • • Ultrasound/Doppler: Useful for carotid/subclavian artery evaluation 

• • • PET-CT: Detects active vessel inflammation (especially in early disease) 

Additional Testing: 

• • Echocardiogram: Assess aortic regurgitation, LV hypertrophy 

• • Renal Doppler: Evaluate for renal artery stenosis in hypertensive patients 

• • CBC: Mild normocytic anemia may be present 

• • Autoimmune panel: ANA, ANCA to rule out other vasculitides 

Treatment

I) Medical Management:

Immunosuppression: 

• • Glucocorticoids: First-line (e.g., prednisone 1 mg/kg/day), tapered over months 

• • Steroid-sparing agents: 

• • Methotrexate 

• • Azathioprine 

• • Mycophenolate mofetil 

• • Biologic agents: 

• • Tocilizumab (IL-6 inhibitor) 

• • TNF inhibitors (e.g., infliximab) for refractory disease 

Cardiovascular Risk Management: 

• • Antihypertensives (e.g., ACE inhibitors for renal artery involvement) 

• • Statins if dyslipidemia present 

• • Antiplatelet therapy (aspirin) for stroke prevention 

II) Interventional/Surgical:

• • Revascularization: Indicated for critical stenosis or symptomatic occlusion 

• • Percutaneous angioplasty ± stenting 

• • Surgical bypass grafting 

• • Aortic valve replacement: For severe aortic regurgitation 

• • Surgery ideally performed during remission phase to reduce complications 

Patient Education, Screening, Vaccines

• • Importance of medication adherence (especially during steroid tapering) 

• • Monitor for early signs of relapse (fatigue, claudication, elevated ESR/CRP) 

• • Blood pressure monitoring in both arms 

• • Routine ophthalmologic exams 

• • Avoid tobacco use 

• • Vaccinations (due to immunosuppression): 

• • Influenza annually 

• • Pneumococcal vaccine 

• • Hepatitis B (if immunosuppressive therapy planned) 

• • COVID-19 vaccination 

Consults

• • Rheumatology: Central for diagnosis and long-term management 

• • Vascular Surgery: For planning revascularization or bypass 

• • Nephrology: In cases of renal artery stenosis or hypertension 

• • Cardiology: If aortic regurgitation or coronary involvement suspected 

• • Ophthalmology: For visual symptoms 

• • Infectious Disease: If tuberculosis is suspected or biologics are used 

Follow-Up

• • Inflammatory Marker Monitoring: 

• • • ESR and CRP every 1–3 months during active disease or therapy changes 

• • Imaging Surveillance: 

• • • Repeat MRI/MRA or CTA every 6–12 months to monitor vascular progression 

• • Medication Adjustment: 

• • • Steroid tapering guided by clinical and lab response 

• • • Evaluate for steroid-related side effects (osteoporosis, glucose intolerance) 

• • Blood Pressure Control: 

• • • Frequent checks in both arms 

• • • Monitor renal function and electrolytes during ACE inhibitor therapy 

• • Relapse Monitoring: 

• • • Symptoms (fatigue, limb pain, bruits) 

• • • Inflammatory markers 

• • • Imaging for new lesions