Update on therapeutic approaches in ANCA-associated vasculitis

Area of Science:

• Rheumatology
• Immunology
• Pharmacology

Background:

• Standard-of-care for antineutrophil cytoplasmic antibody-associated vasculitis (AAV) relies on glucocorticoids (GCs) and immunosuppressants like rituximab.
• These treatments exhibit partial efficacy, significant toxicity, and risk of relapse, impacting patient outcomes.
• Optimized GC dosing and the introduction of avacopan offer alternatives, but further advancements are needed.

Purpose of the Study:

• To review the current landscape of AAV treatment.
• To highlight the limitations of existing therapies.
• To discuss emerging therapeutic targets and drug development in AAV.

Main Methods:

• Review of current literature on AAV standard-of-care.
• Analysis of clinical trial data for optimized GC dosing and avacopan.
• Exploration of recent research on AAV pathophysiology and drug targets.

Main Results:

• Glucocorticoids and rituximab remain mainstays but are associated with toxicity and relapse.
• Avacopan provides an alternative to GCs, and optimized dosing strategies are established.
• Advances in understanding AAV pathogenesis reveal new targets, including cytokines, complement, and B cells.

Conclusions:

• Despite advances, AAV treatment requires improved strategies to enhance efficacy and minimize toxicity.
• Targeting specific inflammatory and immune pathways holds promise for novel AAV therapeutics.
• Ongoing research into cytokine, complement, and B cell targets is crucial for developing next-generation AAV treatments.