Emerging treatments
Janus kinase (JAK) inhibitors
JAK inhibitors (e.g., tofacitinib, upadacitinib) inhibit the activity of one or more JAK enzymes and block signaling of cytokines implicated in Takayasu's arteritis pathogenesis (type 1 and 2 interferons, interleukin [IL]-6, IL-12, IL-17, and IL-23). They also suppress tissue-resident memory T cells and reduce inflammatory-related vascular damage.[48][49][50] Case reports and one prospective observational study show positive results in patients with Takayasu's arteritis (clinical improvement, lower radiological disease progression, superior to methotrexate) for tofacitinib. There is a current ongoing randomised controlled trial now in phase 4 comparing methotrexate and tofacitinib.[51] There is one ongoing RCT evaluating the efficacy of upadacitinib in combination with corticosteroids, compared with placebo and corticosteroids.[52] JAK inhibitors are not included in the 2018 EULAR recommendations or the 2021 ACR guidelines.
Abatacept
T-cell-mediated mechanisms are involved in the pathogenesis of Takayasu's arteritis. Abatacept inhibits activation of T cells. However, in one randomised clinical trial, addition of abatacept to a treatment regimen with prednisolone (prednisone) did not reduce the risk of relapse in patients with Takayasu's arteritis.[53] Abatacept is not currently recommended in guidelines due to lacking evidence for efficacy.[3]
Secukinumab
Secukinumab is a human monoclonal antibody that selectively binds to interleukin 17A (IL-17A). IL-17 has been implicated in the pathogenesis of Takayasu's arteritis, as raised levels of this cytokine and expansion of Th17 cells are seen in patients with this disease.[18] A 2023 trial evaluated the efficacy of secukinumab in patients with refractory Takayasu's arteritis. In a single-centre open-label trial, 19 patients were treated with secukinumab and compared with 34 patients treated with tumour necrosis factor (TNF)-alpha inhibitors. The overall efficacy of secukinumab was similar to that of TNF-alpha inhibitors with complete response and partial response rates of 52.6% and 64.7%, respectively, at 6 months.[54] While encouraging, these results require future validation in randomised trials.
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