Avacopan improves treatment response in refractory ANCA-associated renal vasculitis with concominant complement system activation. (a) Time of kidney biopsies and treatment regimens. (b,c) Time course of serum creatinine and uACR levels. (d,e) Representative sections from the first and second kidney biopsy stained with periodic acid-schiff confirmed focal class necrotizing and crescentic ANCA-associated renal vasculitis. (f) Measurements of serum C3c and C4 at time of first and second kidney biopsy, the dotted lines represent the lower limit of the normal range. (g) Representative sections from the first and second kidney biopsy stained for intrarenal complement C3c confirmed progressive deposits (scale bar: 200 μm). ANCA, antineutrophil cytoplasmic antibody; KRT, kidney replacement therapy; PAS, periodic acid-schiff; uACR, Urine albumin to creatinine ratio.