Vasculitis therapy refines vasculitis mechanistic classification

The primary vasculitides constitute a heterogeneous group of immune mediated diseases incompletely understood pathogenesis currently classified by the size blood vessels affected (Chapel Hill classification). In recent years, several drugs with well-characterized immunological targets have been tested in clinical trials large vessel vasculitis and small vasculitis. Such provide reverse translational or bedside to bench information about underlying pathogenic mechanisms. Therefore, aim this systematic literature review was examine evidence base for more refined mechanistic classification A total 40 studies (20 randomized controlled (RCTs), 16 prospective studies, 1 retrospective cohort study 3 case series) were included full qualitative assessment. RCTs concerning biologic therapy mainly supports interleukin 6 receptor inhibition (tocilizumab). granulomatosis polyangiitis microscopic support anti-CD20 treatment (rituximab) complement molecule C5a antagonist (avacopan) is an emerging option. eosinophilic centered around 5 (mepolizumab). Studies on tumor necrosis factor alpha (adalimumab, infliximab, etanercept) showed negative results giant cell arteritis but some effect Takayasu arteritis. Taken together, cytokine specific are dissecting immunopathogenic mechanisms classification. Especially, antagonism pointing towards distinctions between polyangiitis/microscopic differences