*2.6.1 Corticosteroids*

First-line treatment. Children with corticosteroid resistance should receive high-dose corticosteroids combined with another immunosuppressant agent, usually methotrexate, in order to avoid irreversible vessel damage [11].

## *2.6.2 Immunosuppressant drugs*

Can be used in children as first or second-line agents. Has been shown to be safe and effective as a second agent to achieve sustained remission, decrease steroid dose and improve vascular lesions. This drug include methotrexate, cyclophosphamide, azathioprine, and mycophenolate mofetil [11].

### *2.6.3 Biological agents*

More than one-half (54%) of the patients required treatment with biological agents. Antitumor necrosis factor agents (infliximab, etanercept, and adalimumab), and anti-IL-6 therapy (tocilizumab) have been used with variable effectiveness [11].

Some evidence from the case series suggests that infliximab may be effective in the management of refractory Takayasu arteritis, but has been shown to be effective in inducing and maintaining remission [7, 11].

Due to the fact that in various studies there are theories about the importance of IL-6 in the pathogenesis of this condition, such as the increase in IL-6 expression, blockade with tocilizumab has been shown to be effective in children [7].

Due to the role of Th1 and Th17 cells in the pathogenesis of the disease, there are some published cases with the use of ustekinumab (anti-IL-12/23) [7].
