Background Takayasu arteritis (TA) is a rare chronic-relapsing granulomatous vasculitis involving primarily the aorta and its major branches¹. TA is associated with considerable morbidity and mortality². Therapy is based on corticosteroids (CS) but steroid-sparing immunosuppressive drugs are required in most patients to minimize CS adverse events and to control progressive vascular disease¹. However, about 25% of patients relapse when CS are tapered³. In this setting, previous works showed that anti-TNF (aTNF) drugs may be useful, with a response rate of about 60-70%^4,5.

Objectives To evaluate safety and efficacy of aTNF drugs in the treatment of refractory TA.

Methods We retrospectively studied 15 TA patients treated with aTNF drugs between 2004 and 2011 at a single academic Italian center. All patients satisfied ACR criteria for TA classification.

Results 15 female patients (mean age at the beginning of aTNF therapy 36 years) with active relapsing TA were studied. All had been treated with at least one immunosuppressive drug and CS. Mean follow-up (FU) was 46 months (range 11-56). Mean duration of CS therapy was 37 months before aTNF. 13 patients received infliximab, 5 adalimumab and 1 golimumab. Mean prednisone (PDN) daily dose could be reduced from 17,8 mg to 7,9 mg at the end of FU (p0,036). None of the patients could be weaned off PDN. A reduction in both ESR and CRP was observed (respectively from 39 to 26 mm/h and from 19.5 to 7.1 mg/l, p 0,011 and 0,003). At the end of FU 27% of patients showed active disease according to NIH criteria³. Vascular involvement was assessed by angio-MR in 14 cases and by echography in only one subject: the mean number of lesions per patient reduced from 9,9 to 7,7 (p0,002). At vascular sites not previously treated with a revascularisation procedure (native lesions) 53% of lesions improved and only 11% showed progressive worsening, while revascularised lesions showed progressive worsening in 67% of vascular sites (even in patients whose other native vascular lesions improved). Partial response (i.e. PDN daily dose reduction ≥50%) was obtained in 5 cases; complete sustained response (i.e. absence of imaging progression and features of active disease while on PDN daily dose ≤7.5 mg for at least 6 months) in 8. No serious side effects, including infection, were observed during FU and none of the patients discontinued aTNF therapy due to adverse events.

Conclusions According to previous studies^4,5, we confirmed that aTNF drugs could be considered as an effective and safe steroid sparing option for refractory TA. However, even if we observed a significant improvement of native vascular lesions, we documented a very low efficacy in preventing revascularisation failure, suggesting that the pathophysiology of revascularised lesions and native lesions could differ.

1. Mason JC. Takayasu arteritis-advances in diagnosis and management. Nat Rev Rheumatol 2010;6:406-15

2. Maksimowicz MKK et al. Takayasu arteritis: what is the long term prognosis? Rheum Dis Clin N Am 2007;33:777-86

3. Kerr GS et al. Takayasu arteritis. Ann Int Med 1994;120:919-29

4. Hoffman GS et al. Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum. 2004;50:2296-304

5. Molloy ES et al. Anti-TNF therapy in patients with refractory Takayasu arteritis: long-term follow-up. Ann Rheum Dis 2008;67:1567-69

Disclosure of Interest None Declared