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. 2024 Jul 6;9(9):2803-2808.
doi: 10.1016/j.ekir.2024.07.007. eCollection 2024 Sep.

Avacopan in Anti-Neutrophil Cytoplasmic Autoantibodies-Associated Vasculitis in a Real-World Setting

Jonas Zimmermann  1   2 , Janis Sonnemann  1   2 , Wolfram J Jabs  3 , Ulf Schönermarck  4 , Volker Vielhauer  4 , Markus Bieringer  5 , Udo Schneider  6 , Ralph Kettritz  1   2 , Adrian Schreiber  1   2
Affiliations

Avacopan in Anti-Neutrophil Cytoplasmic Autoantibodies-Associated Vasculitis in a Real-World Setting

Jonas Zimmermann et al. Kidney Int Rep. .
No abstract available

Keywords: anti-neutrophil cytoplasmic autoantibodies (ANCA); avacopan; complement C5a; necrotizing crescentic glomerulonephritis; vasculitis.

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Figures

Figure 1
Figure 1
(a) Course of individual patients (primary end points, duration of avacopan treatment) and prednisolone tapering during 12-month follow-up. All included patients are shown over time (weeks) and sorted by subgroups (eGFR < 15 ml/min, DAH). Three patients had both eGFR < 15 ml/min and DAH and were assigned to both groups. The black dots at week 26 indicate remission (28/32 patients), and the green dots at week 52 represent sustained remission (21/23 patients). If there are no dots at these time points, remission or sustained remission was not reached. Reasons for not achieving remission include 2 relapses, 1 BVAS > 0, and 1 prednisolone dose > 7.5 mg/d; reasons for not achieving sustained remission include 2 relapses. The black lines represent the duration of avacopan therapy, with arrows indicating ongoing use. Due to the retrospective nature of the analysis, the exact timing of avacopan initiation is unknown and is therefore represented as day 0 in the figure. Red crosses at the end of a black line indicate that an adverse event (AE) caused therapy discontinuation. The dashed line represents the follow-up period. The average duration of avacopan therapy among patients with a ≥52-week follow-up and for the whole group is shown in the black box. Therapy with avacopan was discontinued in 9 patients (23%), of which 8 cases (21%) were due to AEs: fever and leukopenia (2 patients), significant increases in transaminase levels (2 patients), intolerable gastrointestinal side effects (2 patients), severe cough, and respiratory mucus production (2 patients). (b) The average daily oral prednisolone dose (mean ± SD, represented by the red solid line) is shown for the entire study population (n = 39). For comparison, the prednisolone tapering regimen from the PEXIVAS study (reduced dose for 50–75 kg of body weight, mean, depicted by the dashed line) is illustrated. The black line indicates a prednisolone dose of ≤7.5 mg. In addition, the oral prednisolone dose, i.v. methylprednisolone dose, and the total prednisolone equivalent dose are provided. AEs, adverse event; BVAS, Birmingham Vasculitis Activity Score; DAH, diffuse alveolar hemorrhage; eGFR, estimated glomerular filtration rate, PEXIVAS, plasma exchange and glucocorticoids in severe antineutrophil cytoplasmic antibody-associated vasculitis.
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