Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

all patients

1st line – glucocorticoid

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1st line – 

glucocorticoid

Glucocorticoids are the mainstay of therapy to suppress vascular inflammation and systemic inflammatory symptoms. Duration of therapy varies, but dose can be reduced once signs and symptoms have diminished and acute phase markers have normalized.

A common tapering regimen is to reduce prednisone by 5 mg/week until reaching a dose of 20 mg/day. Thereafter, the taper rate is decreased to 2.5 mg/week until reaching a dose of 10 mg/day. Thereafter, the dose is lowered by 1 mg/day each week, as long as disease does not become more active.[8] The 2018 European League Against Rheumatism (EULAR) recommendations for management of large vessel vasculitis suggest reducing prednisone to a dose of 15-20 mg/day within 2 to 3 months, and then to ≤10 mg/day after 1 year.[38]

Glucocorticoid tapering may have to be stopped and the dose increased if there is return of disease activity.

Long-term glucocorticoid therapy increases the risk of osteoporosis, and the greatest amount of bone loss occurs in the first 6-12 months of therapy. Risk is proportional to cumulative glucocorticoid dose, so dose should be reduced as soon as possible. It is important to prevent bone loss by ensuring adequate dietary calcium intake and prophylactic use of bisphosphonates and vitamin D3/calcium supplementation. See Osteoporosis.

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. See Pneumocystis jirovecii pneumonia.

Primary options

prednisone: 1 mg/kg/day orally initially, then taper according to response, usual starting dose 40-60 mg/day

Plus – immunosuppressant

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Plus – 

immunosuppressant

Treatment recommended for ALL patients in selected patient group

Relapse occurs in more than 50% of patients during glucocorticoid tapering. Nonglucocorticoid immunosuppressive agents are recommended in addition to glucocorticoids over monotherapy with glucocorticoids to minimize glucocorticoid-related toxicity.[3]​ Methotrexate is often used as the initial agent, but other therapies such as azathioprine, leflunomide, or mycophenolate can be considered as well. Glucocorticoid monotherapy can be considered for mild disease or if the diagnosis is uncertain.[3][37][38]

In an open-label study, methotrexate was effective as a corticosteroid-sparing agent for a subset of patients with Takayasu arteritis.[37]

Azathioprine or mycophenolate or leflunomide can be considered for patients who are intolerant to, or relapse while on, methotrexate.[8][48]

In patients with severe or life- or organ-threatening disease, use of cyclophosphamide may be indicated. However, the benefit of 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.

Primary options

methotrexate: 15-25 mg orally/subcutaneously once weekly on the same day each week

-- AND --

leucovorin: 10 mg orally every 6 hours for 10 doses starting 10 hours after methotrexate dose

or

folic acid (vitamin B9): 1 mg orally once daily

Secondary options

azathioprine: 2 mg/kg/day orally

OR

mycophenolate mofetil: 1 to 1.5 g orally twice daily

OR

leflunomide: 20 mg orally once daily

Tertiary options

cyclophosphamide: 2 mg/kg/day orally

Consider – low-dose aspirin

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Consider – 

low-dose aspirin

Treatment recommended for SOME patients in selected patient group

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]

If used, aspirin is usually stopped 1 week prior to any surgical procedure.

Primary options

aspirin: 75 mg orally once daily

Consider – biologic agent

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Consider – 

biologic agent

Treatment recommended for SOME patients in selected patient group

Tumor necrosis factor (TNF)-alpha antagonist therapy may be considered with persistent active disease despite treatment with glucocorticoids and immunosuppressive agents.

In a pilot study of relapsing Takayasu arteritis, anti-TNF therapy resulted in improvement in 93% of patients and sustained remission in 67%. Most patients in this study were treated with infliximab.[40]

Retrospective reviews also support the use of anti-TNF therapy for refractory disease.[41][42]

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]

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

Primary options

infliximab: 3-5 mg/kg intravenously as a single dose at 0, 2, and 6 weeks, then every 4-8 weeks thereafter

Secondary options

tocilizumab: 6 mg/kg intravenously every 4 weeks, maximum 600 mg/dose; 162 mg subcutaneously once weekly

Consider – evaluation for surgery or endovascular procedure

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Consider – 

evaluation for surgery or endovascular procedure

Treatment recommended for SOME patients in selected patient group

Vascular lesions are not usually reversible with immunosuppression alone. Therefore, patients with significant limb claudication or severe ischemic organ dysfunction may require surgical intervention. Some reports have indicated good short-term outcomes with percutaneous intervention for vascular stenoses in patients with Takayasu arteritis. However, restenoses are common with this intervention, and bypass surgery often yields better long-term results.[8][44]

Patients with aneurysmal disease 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. Patients with active disease requiring operation are more likely to require revision.[45] Therefore, control of inflammation prior to vascular surgery is important to improve surgical outcome. Long-term complications may include anastomotic aneurysms and congestive heart failure.[47]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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