Mapping a path forward: addressing disease burden, pathways and solutions in ANCA-associated vasculitis

Area of Science:

• Rheumatology
• Immunology
• Internal Medicine

Background:

• Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a severe autoimmune disease causing small- to medium-sized blood vessel inflammation and organ damage.
• Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are the most prevalent AAV subtypes, often characterized by frequent relapses.

Purpose of the Study:

• To summarize current international and national guidelines for treating GPA and MPA.
• To highlight the importance of reducing treatment-related adverse events in AAV management.

Main Methods:

• Review of existing international and national treatment guidelines for GPA/MPA.
• Analysis of recommended induction and maintenance therapy strategies.

Main Results:

• Guidelines prioritize rapid disease control using induction therapy (rituximab or cyclophosphamide with glucocorticoids and avacopan).
• Emphasis is placed on reducing glucocorticoid (GC) exposure through rapid tapering and utilizing GC-sparing agents like avacopan.
• Maintenance strategies aim to minimize relapse risk.

Conclusions:

• Current guidelines for GPA/MPA treatment are largely consistent, focusing on efficacy and safety.
• Reducing GC toxicity via avacopan and rituximab is a key therapeutic goal.
• Ongoing research is exploring novel GC-sparing treatments and optimizing rituximab regimens for AAV.