Guidelines Summary
The 2021 American College of Rheumatology/Vasculitis Foundation (ACR/VF) guideline includes recommendations on the following aspects of managing Takayasu arteritis (TAK) [52] :
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Medical therapy
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Monitoring of disease activity
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Vascular imaging
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Surgical intervention
Medical management
ACR/VF recommendations regarding medical therapy include the following [52] :
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For patients with active, severe TAK who are not receiving immunosuppressive therapy, consider initial treatment with high-dose oral glucocorticoids; IV pulse glucocorticoids followed by high-dose oral glucocorticoids may be considered for patients with life- or organ-threatening disease. In children, alternative steroid dosing regimens (eg, IV pulse glucocorticoids with low daily oral dosing) may be preferred.
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High-dose glucocorticoids are preferred over low-dose glucocorticoids for initial treatment of newly active, severe TAK.
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Once remission is achieved after receiving glucocorticoids for ≥6–12 months, tapering off glucocorticoids is preferred over long-term treatment with low-dose glucocorticoids for remission maintenance.
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A nonglucocorticoid immunosuppressive agent plus glucocorticoids is preferred over glucocorticoids alone for treatment of active TAK.
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Nonglucocorticoid immunosuppressive therapy is preferred over tocilizumab as initial therapy for active TAK.
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For patients with TAK that is refractory to treatment with glucocorticoids alone, adding a tumor necrosis factor inhibitor is preferred over adding tocilizumab.
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For active TAK with critical cranial or vertebrobasilar involvement, aspirin or another antiplatelet therapy may be added.
In addition, medical management is recommended over surgical management for patients with TAK that involves the following:
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Renovascular hypertension and renal artery stenosis
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Stenosis of a cranial/cervical vessel without clinical symptoms
In patients with TAK and worsening signs of limb/organ ischemia while receiving immunosuppressive therapy, escalating immunosuppressive therapy is preferred over surgical intervention with escalation of immunosuppressive therapy. Surgery is not recommended for patients with persistent limb claudication without evidence of ongoing active TAK. [52]
For patients undergoing surgical intervention, high-dose glucocorticoids should be given periprocedurally if the patient has active disease.
Monitoring of disease activity
ACR/VF recommendations regarding monitoring in patients with TAK include the following [52] :
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Consider adding inflammation markers to clinical monitoring, to assess disease activity.
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Long-term clinical monitoring is strongly recommended for patients in apparent clinical remission.
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For patients with TAK in apparent clinical remission but with an increase in levels of inflammation markers, consider clinical observation without escalation of immunosuppressive therapy, as such increases can be nonspecific.
Vascular imaging
ACR/VF recommendations regarding vascular imaging for patients with TAK include the following [52] :
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Consider the use of noninvasive imaging (eg, computed tomography angiography [CTA], magnetic resonance angiography [MRA], or fluorodeoxyglucose positron emission tomography [FDG-PET]) over catheter-based dye angiography for assessment of disease activity.
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Consider regularly scheduled noninvasive imaging (eg, every 3–6 months or longer) in addition to routine clinical assessment.
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For patients in apparent clinical remission but with signs of inflammation in new vascular territories (eg, new stenosis or vessel wall thickening) on vascular imaging, consider immunosuppressive therapy.
Surgical intervention
In an ungraded position statement, the ACR/VF advises that for any patient requiring surgical vascular intervention, the type and timing of intervention should be a collaborative decision between the vascular surgeon and rheumatologist. The ACR/VF guideline provides recommendations regarding surgical intervention for patients with known TAK and the following manifestations [52] :
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Persistent limb claudication without evidence of ongoing active disease - Conditionally recommend against surgical intervention
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Worsening signs of limb/organ ischemia while receiving immunosuppressive therapy - Conditionally recommend escalating immunosuppressive therapy over surgical intervention with escalation of immunosuppressive therapy
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Renovascular hypertension and renal artery stenosis - Conditionally recommend medical management over surgical intervention
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Stenosis of a cranial/cervical vessel without clinical symptoms - Conditionally recommend medical management over surgical intervention.
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Worsening signs of limb/organ ischemia - Conditionally recommend delaying surgical intervention until the disease is quiescent over performing surgical intervention while the patient has active disease.
For patients with active TAK who are undergoing a vascular surgical intervention due to a complication of the disease, the guideline conditionally recommends periprocedure use of high-dose glucocorticoids.
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Takayasu arteritis. Complete occlusion of the left common carotid artery in a 48-year-old woman with Takayasu disease. Also note narrowing of the origin of the right subclavian artery and a narrowed small vessel with subsequent aneurysmal dilatation on the right side. Image courtesy of Robert Cirillo, MD.
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Takayasu arteritis. Characteristic long, tapered narrowing of the distal aorta and iliac vessels. Image courtesy of Robert Cirillo, MD.
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Takayasu arteritis. Image obtained in the same patient as in Image 2 reveals narrowing of the proximal descending aorta and right brachiocephalic artery. Image courtesy of Robert Cirillo, MD.
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Takayasu arteritis. Aortogram of a 15-year-old girl with Takayasu arteritis. Note large aneurysms of descending aorta and dilatation of innominate artery. Image courtesy of Christine Hom, MD.
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Takayasu arteritis. MRI of thorax of 15-year-old girl with Takayasu arteritis. Note aneurysms of descending aorta. Image courtesy of Christine Hom, MD.
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Takayasu arteritis. Coronal MRI of abdomen of 15-year-old girl with Takayasu arteritis. Note thickening and tortuosity of abdominal aorta proximal to kidneys. Image courtesy of Christine Hom, MD.
