Advances in the treatment of ANCA-associated vasculitis
A review of how treatment of ANCA-associated vasculitis has shifted: rituximab is now established for both remission induction and maintenance, steroid doses have been cut substantially without losing efficacy, and the oral C5a receptor antagonist avacopan has been approved as an add-on. Newer agents including anti-CD19 CAR T cells and next-generation complement inhibitors are under investigation.
Background
ANCA-associated vasculitis comprises a group of small-vessel vasculitides that frequently present with organ-threatening or life-threatening disease, commonly including rapidly progressive glomerulonephritis. Current immunosuppressive regimens have improved survival and remission rates but are not curative, carry frequent toxicities, and do not reliably prevent relapse.
Key findings
Clinical trials have established rituximab, an anti-CD20 B-cell-depleting monoclonal antibody, in both the remission-induction and maintenance phases. Trials have also demonstrated that glucocorticoid doses can be substantially reduced from historical levels without compromising efficacy. Avacopan, an oral C5a receptor antagonist, has been approved as an adjunctive treatment; used with rituximab or cyclophosphamide alongside markedly reduced glucocorticoid dosing, it demonstrated superior efficacy and potentially greater kidney recovery than prior standard of care.
Clinical implications
The review positions B-cell depletion as the therapeutic backbone, with glucocorticoid minimisation now supported rather than merely tolerated, and complement blockade as an established adjunct. Agents under study include next-generation anti-CD20 monoclonal antibodies, anti-CD19 chimeric antigen receptor T cells, novel complement inhibitors, and agents targeting fibrosis. Alongside traditional randomised trials with clinical endpoints, experimental medicine studies are increasingly focused on mechanistic endpoints and disease biomarkers.
Safety
The review emphasises that existing regimens carry frequent toxicities, which motivates both the glucocorticoid-reduction strategy and the search for more targeted agents. Demonstrating that lower glucocorticoid exposure does not cost efficacy is presented as one of the more clinically consequential safety advances in the field.
Category
Research
Source
Nature Reviews Rheumatology
More from ASNRT News
Browse the latest news, society announcements, KDIGO guideline updates, and AJNT issue releases on the ASNRT newsroom.