PURPOSE OF REVIEW: Vasculitides can affect small, medium and/or large vessels, 
leading to end-organ damage, decreased quality of life and death. 
Glucocorticoids remain the backbone of treatment for systemic vasculitis but are 
associated with numerous toxicities. In recent years, the efficacy of 
glucocorticoid-sparing biologic and novel small molecule therapies has been 
demonstrated.
RECENT FINDINGS: In giant cell arteritis, tocilizumab was superior to 
glucocorticoid monotherapy in maintenance remission and cumulative 
glucocorticoid exposure and is now approved for the treatment of giant cell 
arteritis. In addition to the previously demonstrated efficacy of rituximab for 
remission induction in antineutrophil cytoplasmic antibody (ANCA)-associated 
vasculitis, recent trials have also demonstrated its superiority for remission 
maintenance compared to alternative approaches. Mepolizumab is superior to 
standard of care alone with regard to remission rates and glucocorticoid-sparing 
effect in refractory eosinophilic granulomatosis with polyangiitis. Avacopan has 
shown significant promise in ANCA-associated vasculitis as part of a 
glucocorticoid-free induction regimen in a recently completed phase 3 trial. Use 
of biologics in rarer vasculitides remains guided by reports from small case 
series.
SUMMARY: Biologics and other novel therapies have an increasingly important role 
in the management of systemic vasculitis. Additional studies are needed to 
define their optimal use and to guide their use in more rare forms of 
vasculitis.