Extrarenal Disease in ANCA-Associated Vasculitis: Challenges and Evolving Therapeutic Strategies on behalf of the Immunonephrology Working Group (IWG) of the ERA - PubMed
6 hours ago
- #therapeutic strategies
- #ANCA-associated vasculitis
- #extrarenal manifestations
- ANCA-associated vasculitis (AAV) encompasses systemic small-vessel necrotizing vasculitides, including MPA, GPA, and EGPA.
- Genetic factors, such as HLA variants, influence disease susceptibility and define distinct MPO and PR3 associated phenotypes.
- Kidney involvement is common, particularly in MPA, while GPA and EGPA frequently present with extrarenal manifestations like pulmonary, ENT, neurological, and cutaneous issues.
- Current treatments focus on rituximab or cyclophosphamide with reduced-dose glucocorticoids for induction, avacopan for glucocorticoid-sparing, and rituximab for maintenance.
- Avacopan shows benefits beyond renal outcomes, and anti-interleukin-5 therapies are now approved for EGPA, but ILD remains a therapeutic challenge.
- AAV patients face high risks of infections, thromboembolism, and malignancy, with mortality driven mainly by infections, and kidney and lung involvement are key prognostic factors.