Advances and challenges in management of large vessel vasculitis.

ABSTRACT

Glucocorticoids (GC) remains the mainstay for management of large vessel vasculitis (LVV). Recent introduction of interleukin-6 signaling blocker, tocilizumab has substantially changed the practice in management of patients with LVV, in particular, giant cell arteritis (GCA). Benefit of tocilizumab to patients with Takayasu arteritis (TAK) is supported by observational studies, but randomized clinical trials are lacking. Addition of tocilizumab enables reduction of the total amount of GC in patients with GCA, but GC burden remains high and to be further reduced. Ongoing studies aim at minimal use of GC or even GC-free. Tumor necrosis factor inhibitors appear to be beneficial to TAK despite their ineffectiveness to GCA. Randomized clinical trials are undergoing to target other inflammatory cytokines in both GCA and TAK. Janus kinase inhibitors alone or in combination with conventional disease modifying anti-rheumatic drugs showed promising results in treatment of TAK.