2020 Volume 3 Issue 2 Pages 279-281
Primary vasculitis is classified according to vessel size in 2012 revised international Chapel Hill consensus conference nomenclature；Takayasu arteritis（TAK）and giant cell arteritis（GCA）were classified into large vessel vasculitis（LVV）and ANCA associated vasculitis（AAV）were classified as small vessel vasculitis different from immune complex related vasculitis.
LVV has been treated with glucocorticoids（GCs）and various immunosuppressants, but they are often refractory. Recently, some studies reported the efficacy of interleukin-6-receptor inhibitor, tocilizumab（TCZ）, for LVV. GiACTA study showed that the efficacy of remission maintenance in GCA patients without GCs, while TAKT study reported the efficacy of the prevention of relapse in the refractory TAK patients.
For AAV, rituximab is emerged as the alternative option to cyclophosphamide and azathioprine for remission induction and maintenance treatments. Additionally, recent report suggested GCs sparing effect of C5a receptor inhibitor, avacopan.
（日本皮膚免疫アレルギー学会雑誌, 3（2）：279-281, 2020）
