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Takayasu arteritis (aortic arch syndrome) is a systemic vasculitis of large vessels characterized by granulomatous inflammation of the aorta and its major branches. It most commonly affects Asian women < 40 years of age. Patients typically present with constitutional symptoms, decreased bilateral brachial and radial pulses (the disease is also known as pulseless disease), angina, and syncope. Imaging (e.g., MR angiography) is used to confirm the diagnosis; findings include vascular wall thickening and contrast enhancement, and luminal stenosis or occlusion. Biopsy of the affected vessels can also confirm the diagnosis but is not routinely indicated. Management typically involves immunosuppressive agents (e.g., high-dose glucocorticoids plus methotrexate); surgical therapy (e.g., vascular bypass) is indicated for patients with limb ischemia.
- Nonspecific symptoms: Fever, malaise, arthralgia, night sweats
- Vascular symptoms
- Skin manifestations
You can't TAKe a pulse in TAKayasu arteritis (pulseless disease).
General principles 
- Diagnosis is primarily clinical but always requires confirmation with imaging or biopsy.
- Nonspecific laboratory findings may support the diagnosis: e.g., ↑ ESR, ↑ CRP, leukocytosis, anemia 
- Serial measurements of CBC and inflammatory markers may be used for follow-up.
Imaging studies 
Imaging of the aorta and major branches involves:
MR angiography (MRA)
- Indications: preferred initial study for all patients
- Thickening and contrast enhancement of the vascular wall (i.e., inflammation)
- Luminal stenosis or occlusion
- Echocardiography: Consider for all patients to assess for aortic regurgitation and ventricular hypertrophy.
Diagnostic confirmation frequently relies on imaging, as biopsy is not always possible because affected vessels are often inaccessible. 
Biopsy of affected vessel 
General principles 
- Consult rheumatology for all patients.
- Pharmacotherapy with immunosuppressants is the mainstay of treatment.
- Regimens are guided by disease severity and activity.
- Goal is to achieve and maintain clinical remission
- Surgical therapy is reserved for selected cases.
- Initial therapy: Start remission induction therapy for patients with active disease.
- Subsequent therapy: based on response to initial therapy
Glucocorticoid monotherapy is associated with a high relapse rate (∼ 70%). 
Surgical therapy 
Surgical interventions include:
- Vascular bypass
- Vascular graft
- Stent placement
Except for emergency indications, surgical intervention should be delayed until the patient is stable to decrease the risk of complications and achieve higher patency rates. 
- Clinical evaluation (e.g., every 3–6 months )
- Regular imaging with noninvasive studies (e.g., MRA, CTA)
- Consider monitoring with inflammatory markers.
- Educate all patients on . 
- Monitor for .
- Consider .