Management of Takayasu arteritis: a systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis

Abstract

ObjectiveTo collect available evidence on management of large vessel vasculitis to inform the 2018 update of the EULAR management recommendations.MethodsTwo independent systematic literature reviews were performed, one on diagnosis and monitoring and the other on drugs and surgical treatments. Using a predefined PICO (population, intervention, comparator and outcome) strategy, Medline, Embase and Cochrane databases were accessed. Eligible papers were reviewed and results condensed into a summary of findings table. This paper reports the main results for Takayasu arteritis (TAK).ResultsA total of 287 articles were selected. Relevant heterogeneity precluded meta-analysis. Males appear to have more complications than females. The presence of major complications, older age, a progressive disease course and a weaker inflammatory response are associated with a more unfavourable prognosis. Evidence for details on the best disease monitoring scheme was not found. High-quality evidence to guide the treatment of TAK was not found. Glucocorticoids are widely accepted as first-line treatment. Conventional immunosuppressive drugs and tumour necrosis factor inhibitors were beneficial in case series and uncontrolled studies. Tocilizumab failed the primary endpoint (time to relapse) in a randomised controlled clinical trial; however, results still favoured tocilizumab over placebo. Vascular procedures may be required, and outcome is better when performed during inactive disease.ConclusionsEvidence to guide monitoring and treatment of patients with TAK is predominantly derived from observational studies with low level of evidence. Therefore, higher-quality studies are needed in the future.

Takayasu arteritis: a cohort of Italian patients and recent pathogenetic and therapeutic advances

“…However, in a minority of patients with relapsing/refractory TAK, pro-inflammatory cytokines such as TNF-α, IL-6, IL-17, and IFN-γ likely exert a pathogenic role and should therefore be therapeutically addressed. This is the goal of TNF-α inhibitors, such as etanercept, infliximab, adalimumab, and certolizumab [8,[62][63][64], as well as anti-interleukin (IL)-6 receptor monoclonal antibodies such as tocilizumab [65][66][67][68], which are increasingly being administered. However, controlled clinical studies in a suitable number of patients are required to allow definitive conclusions about the efficacy of these agents.…”

Treatment of Giant Cell Arteritis and Takayasu Arteritis—Current and Future

“…New structural vascular lesions were observed in 20% of patients over the long term. In indirect comparison with other cohorts without early GC-sparing therapy, where relapses and/or new structural lesions occur in up to 80% of patients [3], this suggest a clinically relevant benefit of adding a csDMARD in newly diagnosed TAK patients, as pointed out by the EULAR recommendation of a first-line use of csDMARDs in TA [10].…”

“…However, patients with TA should be managed by a specialist team with access to the multidisciplinary infrastructure and experience of a vasculitis center. Treatment recommendations for TAK are largely based on low-quality evidence retrieved from observational studies, whereas RCTs have only published on the use of TCZ and abatacept [3].…”

“…Around 60-80% of TA patients treated with GC monotherapy relapse [64,65,67]. In addition, many patients develop new vascular lesions and/or cannot reduce the GC dose to an acceptable level [3]. Therefore, EULAR experts recommend an early initiation of GC-sparing therapy.…”

Management of cardiac manifestations in Takayasu arteritis

“…Abatacept (CTLA4-Ig) use in TA did not meet the primary endpoints in randomized controlled trials (RCTs). Rituximab (anti-CD 20) and ustekinumab (anti-IL-12/23) in TA gave variable results in isolated case reports [32][33][34] . High-dose prednisolone (1 mg/kg/day) or its equivalent is the initial therapy for active TA with tapering dose for 3 to 6 months.…”