Abstract
Background Giant cell arteritis (GCA) and Takayasu's arteritis (TAA) are large vessel vasculitides (LVV) for which corticosteroids (CS) are the mainstay for treatment. In patients with LVV unable to tolerate CS, biological agents have been used with variable effectiveness.ObjectiveTo systematically review the effectiveness and safety of biological agents in patients with LVV.MethodsWe searched 5 electronic databases (inception to October 2012) and conference abstracts with no language restrictions. Two reviewers independently selected studies, extracted data and assessed methodological quality. Our protocol was registered in PROSPERO.ResultsWe included 25 studies (3 RCTs and 22 case series with ≥2 cases). 95 GCA and 98 TAA patients received biological agents. The RCTs using anti-TNF agents (infliximab, etanercept and adalimumab) did not suggest a benefit in GCA. GCA patients receiving tocilizumab, in case series, achieved remission (19 patients) and reduction of corticosteroid dose (mean difference, –16.55 mg/day (95% CI: –26.24, –6.86)). In case series, 75 patients with refractory TAA treated with infliximab discontinued CS 32% of the time. Remission was variably defined and the studies were clinically heterogeneous which precluded further analysis.ConclusionThis systematic review demonstrated a weak evidence base on which to assess the effectiveness of biological treatment in LVV. Evidence from RCTs suggests that anti-TNF agents are not effective for remission or reduction of CS use. Tocilizumab and infliximab may be effective in the management of LVV and refractory TAA, respectively, although the evidence comes from case series. Future analytical studies are needed to confirm these findings.
Highlights
Large vessel vasculitis (LVV) includes two major forms, giant cell arteritis (GCA, temporal arteritis) and Takayasu’s arteritis (TAA) [1]
This systematic review identified 25 studies (3 RCTs, 22 case series) of LVV patients treated with five biological agents: IFX, TCZ, ETN, ADA and RXB
Results from case series of patients with Giant cell arteritis (GCA) and TAA suggested that TCZ may be of some benefit for the maintenance of remission, and for the reduction of CS use
Summary
Large vessel vasculitis (LVV) includes two major forms, giant cell arteritis (GCA, temporal arteritis) and Takayasu’s arteritis (TAA) [1]. Both diseases affect mostly females [2] and are defined by inflammatory changes within the walls of the aorta and/or its major branches [3]. Giant cell arteritis (GCA) and Takayasu’s arteritis (TAA) are large vessel vasculitides (LVV) for which corticosteroids (CS) are the mainstay for treatment. In patients with LVV unable to tolerate CS, biological agents have been used with variable effectiveness. Objective: To systematically review the effectiveness and safety of biological agents in patients with LVV. Evidence from RCTs suggests that anti-TNF agents are not effective for remission or reduction of CS use. Tocilizumab and infliximab may be effective in the management of LVV and PLOS ONE | DOI:10.1371/journal.pone.0115026 December 17, 2014
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