Anqi Ni,1– 3 Ying Xu,1– 3 Jianghua Chen,1– 3 Fei Han1– 3 

1Kidney Disease Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, People’s Republic of China; 2Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Zhejiang University, Hangzhou, Zhejiang, People’s Republic of China; 3Institute of Nephrology, Zhejiang University, Hangzhou, Zhejiang, People’s Republic of China

Correspondence: Fei Han, Kidney Disease Center, The First Affiliated Hospital, Zhejiang University School of Medicine, 79 Qingchun Road, Hangzhou, Zhejiang, 310003, People’s Republic of China, Tel +86 57187236996, Email hanf8876@zju.edu.cn

Abstract: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases primarily cause inflammation of small blood vessels. Renal involvement occurs frequently and often leads to end-stage renal disease (ESRD), which significantly impacts patient health and survival. Early diagnosis and appropriate treatment are essential to improving patient outcomes. This review provides an overview of the latest advances in the assessment and treatment of ANCA-associated glomerulonephritis (AAGN). The assessment section covers diagnostic tools, disease activity assessment, and risk stratification, with the introduction of the latest ANCA kidney risk score (AKRiS), which aids in identifying high-risk patients and facilitates personalized treatment strategies. The treatment section discusses induction and maintenance therapy, including immunosuppressive agents and emerging therapies. Recent advances have moved treatment away from reliance on cytotoxic agents, focusing on targeted biological therapies to induce and maintain disease remission, while prioritizing the reduction of corticosteroid toxicity. Overall, these advancements have greatly improved patient outcomes and provided new avenues for personalized management of AAGN.

Keywords: anti-neutrophil cytoplasmic antibody, vasculitis, kidney, assessment, treatment