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Kidney Week

Abstract: FR-PO0643

One Year with Avacopan: A Milan Experience in the Treatment of ANCA-Associated Vasculitis

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Porata, Giulia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardy, Italy
  • Binda, Valentina, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardy, Italy
  • Abinti, Matteo, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardy, Italy
  • Moscardino, Sara, Fondazione IRCCS Policlinico San Matteo, Pavia, Lombardia, Italy
  • Sikharulidze, Anna, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardy, Italy
  • Frontini, Giulia, Azienda Socio Sanitaria Territoriale Santi Paolo e Carlo, Milan, Lombardy, Italy
  • Schioppo, Tommaso, Azienda Socio Sanitaria Territoriale Santi Paolo e Carlo, Milan, Lombardy, Italy
  • Belli, Michele, Fondazione IRCCS San Gerardo dei Tintori, Monza, Lombardy, Italy
  • Masella, Cristina, Azienda Socio Sanitaria Territoriale Grande Ospedale Metropolitano Niguarda, Milan, Lombardy, Italy
  • Mezzina, Nicoletta, Azienda Socio Sanitaria Territoriale Santi Paolo e Carlo, Milan, Lombardy, Italy
  • Frizzarin, Simone, Azienda Socio Sanitaria Territoriale Santi Paolo e Carlo, Milan, Lombardy, Italy
  • Tedesco, Michela, Aziende Socio Sanitarie Territoriale Fatebenefratelli Sacco, Milan, Lombardy, Italy
  • Cozzolino, Mario, Azienda Socio Sanitaria Territoriale Santi Paolo e Carlo, Milan, Lombardy, Italy
  • Gallieni, Maurizio, Aziende Socio Sanitarie Territoriale Fatebenefratelli Sacco, Milan, Lombardy, Italy
  • Pieruzzi, Federico, Fondazione IRCCS San Gerardo dei Tintori, Monza, Lombardy, Italy
  • Minetti, Enrico Eugenio, Azienda Socio Sanitaria Territoriale Grande Ospedale Metropolitano Niguarda, Milan, Lombardy, Italy
  • Castellano, Giuseppe, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardy, Italy
Background

ANCA-associated vasculitis (AAV) is a rapidly progressive autoimmune disease frequently leading to severe renal and pulmonary involvement. Avacopan (AVA), a selective C5a receptor antagonist, has demonstrated efficacy in inducing remission while reducing glucocorticoid (GC) exposure. Since its approval in Italy in mid-2024, AVA has been rapidly adopted in routine clinical practice. We report a multicenter experience from six hospitals in the Milan area, with a focus on safety, renal outcomes, and GC-sparing strategies.

Methods

We evaluated 21 patients (pts) with AAV, predominantly with microscopic polyangiitis (57%) and MPO-ANCA positivity (75%); 76% had biopsy-proven renal disease (Table 1). All pts received induction therapy with AVA and Rituximab. Two pts followed a steroid-free regimen, while 90% received a reduced GC course.

Results

To date, 48% of pts had completed 12 months of AVA therapy. Among these pts, mean eGFR increased by 13.3 mL/min, corresponding to a 63.2% improvement from disease onset. Mean post-therapy follow-up was 4 months [IQR 2.5–15], with no relapses observed. GC withdrawal was achieved in 13/21 pts, with a mean time to discontinuation of 5 months [IQR 2-10] after AVA initiation, reflecting inter-center variability in clinical practice. No pt permanently discontinued AVA due to adverse events. Temporary interruption occurred in 5/21 pts, mainly due to transient elevations in liver transaminases, all of which resolved completely. No severe infections were reported.

Conclusion

In this multicenter real-world cohort, AVA in combination with Rituximab proved highly effective in promoting renal recovery and facilitating rapid GC tapering or complete avoidance. Compared with larger real-world studies, our cohort demonstrated no permanent discontinuations of AVA, no severe infections, and no relapses following treatment withdrawal, supporting a favorable safety profile in a nephrology-focused population. These findings reinforce the role of AVA as a cornerstone of induction therapy in AAV while minimizing steroid-related toxicity.