Tumor necrosis factor alpha inhibitors in patients with Takayasu's arteritis refractory to standard immunosuppressive treatment: cases series and review of the literature

The objective of this study was to report the long-term use of tumor necrosis factor (TNF) inhibitors in case series of patients with Takayasu's arteritis refractory to standard immunosuppressive treatment. Nine women (median age of 29 years) with refractory Takayasu's arteritis were treated with TNF inhibitors. Prior to TNF inhibitor administration, all patients received standard immunosuppressive treatment for 16 to 112 months including steroids and immunomodulators. All but one patient presented with high activity of disease (median ESR 80 mm/h, C-reactive protein level 16.8 mg/l, interleukin-6 level 7.2 pg/ml) that was confirmed with positron emission tomography (PET) with ¹⁸F-deoxyglucose. Eight patients were treated with infliximab and one was treated with adalimumab, respectively. The median duration of treatment was 36 months (12 to 84 months). For induction treatment, we used infliximab 200–300 mg every 4–6 weeks and adalimumab 40 mg every 2 weeks. The treatment resulted in complete remission in five (55.6 %) patients and incomplete remission in three (33.3 %) patients. We were able to taper the dose of prednisone to ≤10 mg daily in all patients. Median levels of ESR, C-reactive protein, and interleukin-6 diminished to 20 mm/h, 1.0 mg/l, and 1.0 pg/ml, respectively. Repeated PET showed lower activity of vasculitis in six (85.7 %) of seven patients. The treatment was safe and well-tolerated. Only one patient developed allergic reactions after infusions of infliximab. Four patients developed relapse of vasculitis when we tried to increase the dosing interval of infliximab to 6–8 weeks. TNF inhibitors were highly effective and safe in patients with refractory Takayasu's arteritis.