Approach

The goal of treatment for Takayasu arteritis is to manage systemic symptoms and suppress vascular inflammation to prevent damage to vessels and the tissues they supply. Glucocorticoids are the mainstay of treatment, with immunosuppressive agents also used for the majority of patients. Surgical or percutaneous revascularization procedures may be required to improve blood flow or prevent rupture of aneurysms. Low-dose aspirin should be considered to help prevent ischemic complications.

Initial presentation

Oral glucocorticoids should be started. No studies have established the optimal way to taper glucocorticoids, but most follow the regimen originally described in the cohort from the National Institutes of Health (NIH).[8]​​[26][36]

Pulse intravenous glucocorticoids have been tried in some patients with central nervous system symptoms and for patients with life- or organ-threatening disease, but there are no data to support their use.[3]

During treatment, patients should be evaluated regularly by clinical examination and measurement of inflammatory markers (i.e., erythrocyte sedimentation rate and C-reactive protein); initially, this may be every few days. Vascular imaging studies such as computed tomography or magnetic resonance angiography should be performed every 3 to 12 months during the active phase of treatment and annually thereafter. Low-dose aspirin should be considered to help prevent ischemic complications.[26]​ Antiplatelet therapy is individualized, taking into account the patient’s risk of ischemic events and risk of bleeding.​​[3][26]

A critical issue is in trying to determine whether or not disease is active. Constitutional symptoms are frequent when Takayasu arteritis is active. However, lack of such symptoms does not mean that the disease is inactive. New vascular lesions can develop even when no other signs, symptoms, or laboratory features of disease activity are present. If new vascular lesions are seen, the disease is considered to be active. Conversely, acute phase markers may be elevated from other causes and not indicate disease activity. Careful evaluation is required.

Corticosteroid-sparing therapy

The majority of patients will go into remission with glucocorticoid therapy. However, relapse occurs in more than 50% of patients during dose tapering. Therefore, European League Against Rheumatism (EULAR) and ACR guidelines recommend early initiation of corticosteroid-sparing immunosuppressive agents such as methotrexate, leflunomide, azathioprine, or mycophenolate.[26][37][38]​​

In patients with severe or life- or organ-threatening disease, use of cyclophosphamide is rarely indicated. However, the benefit of using cyclophosphamide needs to be considered carefully in the context of its known toxicity, particularly premature ovarian failure and long-term risk of secondary malignancies.[39]

There is no evidence to advise on discontinuation of immunosuppressive therapy, and this will depend on individual circumstances.

Refractory to glucocorticoids and additional immunosuppression

Tumor necrosis factor (TNF)-alpha antagonists, primarily infliximab, have also been used successfully in the management of Takayasu arteritis when immunosuppressive agents and glucocorticoid therapy have failed to control disease activity. These agents are used in addition to glucocorticoids as a corticosteroid-sparing agent.[40][41][42]​ ACR guidelines suggest considering the use of a TNF-alpha antagonist as upfront corticosteroid-sparing therapy.[26]

Several small case series of patients treated with tocilizumab, an interleukin (IL)-6 inhibitor, have been reported. In general, most patients have had favorable responses, with corticosteroid-sparing effect, although disease relapse may still occur.[19][43]​ Tocilizumab was evaluated in a double-blind, placebo-controlled trial in patients with refractory Takayasu arteritis. The primary endpoint of the trial (time to relapse) was not met in the intent-to-treat analysis (P=0.06). However, a favorable response was seen in a subset of patients.[20]​ The ACR recommends the use of other nonglucocorticoid immunosuppressive therapy over tocilizumab as initial therapy. It recommends adding a TNF-alpha antagonist over adding tocilizumab in refractory Takayasu arteritis. It recognizes that some practitioners favor TNF inhibition, while others favor IL-6 inhibition in this situation. Overall, the voting panel favored a TNF-alpha antagonist over tocilizumab, since there is more clinical experience with and data on TNF-alpha antagonists in Takayasu arteritis compared to tocilizumab. Tocilizumab should be considered, especially when TNF-alpha antagonists are contraindicated.[3]

​Nonbiologic immunosuppressive agents may be continued or tapered.[43]

Symptoms of intermittent claudication or ischemic organ dysfunction

Surgical intervention may be required in patients with severe complications of Takayasu arteritis. Vascular lesions are usually not reversible with immunosuppression alone. Therefore, patients with significant limb claudication or severe ischemic organ dysfunction may require surgical intervention. Percutaneous angioplasty can be effective in the short term, but restenosis is common.[44] Good long-term outcomes have been reported with vascular bypass surgery.[45][46] Patients with progressive dilation of the aorta may require surgical repair.[47] Except for emergency indications, vascular interventions should preferably be performed when the disease is in remission and inflammation is under control.

Prevention of complications from long-term glucocorticoid use

Prolonged glucocorticoid treatment can lead to significant morbidity. Attention to potential side effects is critical. Long-term glucocorticoid therapy increases the risk of osteoporosis, and the greatest amount of bone loss occurs in the first 6 to 12 months of therapy. Prevention of glucocorticoid-induced bone loss by treatment with calcium, vitamin D, and bisphosphonates is recommended. See Osteoporosis (Management approach).

Glucocorticoid-induced diabetes mellitus is a potential side effect of therapy, and a high index of suspicion is required.

Due to the immunosuppressive effects of glucocorticoids, influenza and pneumococcal vaccination are recommended, and, while the daily prednisone dose is greater than 20 mg/day, prophylaxis for Pneumocystis jirovecii pneumonia is advised.

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