[Update on treatment of ANCA-associated vasculitis]

Julia U Holle1,2, Frank Moosig3,4

  • 1Rheumazentrum Schleswig-Holstein Mitte, Kuhberg 5a-7, 24534, Neumünster, Deutschland. holle@rheuma-sh.de.

PubMed

Insights

Current guidelines recommend biologics like rituximab for ANCA-associated vasculitis (AAV) remission. New treatments include lower-dose glucocorticoids and avacopan, with ongoing research into biomarker-guided therapy.

Area of Science:

  • Rheumatology and Nephrology
  • Immunology and Inflammation
  • Clinical Guideline Development

Context:

  • Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated vasculitis (AAV) management has evolved with new therapeutic options.
  • Established guidelines from EULAR, KDIGO, and ACR inform current treatment strategies.
  • Biologics and targeted therapies are increasingly utilized alongside or replacing traditional immunosuppressants.

Purpose:

  • To summarize and synthesize current international guidelines for managing AAV.
  • To outline the established and emerging treatment modalities for granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA).
  • To identify key areas of unmet need and future research directions in AAV treatment.

Summary:

  • Glucocorticoids (GC) dosing for remission induction in GPA and MPA is reduced.
  • Rituximab (RTX) is a key biologic for remission induction and maintenance in GPA and MPA.
  • Anti-interleukin 5 (IL5) therapies are crucial for refractory/relapsing EGPA.
  • Avacopan offers a novel, GC-sparing option for GPA and MPA over 52 weeks.
  • Conventional agents like cyclophosphamide (CYC) remain vital for severe organ-threatening disease.
  • Methotrexate (MTX) and azathioprine (AZA) use is declining.

Impact:

  • Provides clinicians with an updated overview of AAV treatment recommendations.
  • Highlights the shift towards targeted biologic therapies and reduced glucocorticoid burden.
  • Identifies critical unanswered questions regarding remission maintenance duration and biomarker-based individualized therapy.

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