Division of Kidney Disease and Hypertension, Northwell Health, The Glomerular Disease Center at Northwell Health.
Nephrology Associates of Northern Virginia, Fairfax, VA; Inova Fairfax Hospital, Falls Church, VA.
Adv Kidney Dis Health. 2024 May;31(3):194-205. doi: 10.1053/j.akdh.2024.04.005.
ANCA-associated vasculitis (AAV) is a necrotizing, small-to-medium vessel vasculitis associated with significant morbidity and mortality. AAV is a systemic autoimmune disease affecting kidneys, eyes, sinuses, peripheral nerves, skin, and upper and lower respiratory tracts. AAV tends to present in characteristic phenotypes categorized clinically as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA). Kidney involvement is a common feature of AAV, and has important implications on disease prognosis and management. Existing therapies have been refined and improvements in our understanding of the pathophysiology of AAV has led to approval of novel therapies. In this review, we provide an overview of epidemiology, disease mechanisms, clinical presentation and review therapeutic strategies for induction and maintenance of remission.
抗中性粒细胞胞浆抗体相关性血管炎（AAV）是一种坏死性、小到中型血管炎，与较高的发病率和死亡率相关。AAV 是一种全身性自身免疫性疾病，影响肾脏、眼睛、鼻窦、外周神经、皮肤以及上、下呼吸道。AAV 倾向于以特征性表型表现出来，临床上分为肉芽肿性多血管炎（GPA）、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎（EGPA）。肾脏受累是 AAV 的常见特征，对疾病预后和管理具有重要意义。现有的治疗方法已经得到了改进，对 AAV 病理生理学的认识的提高也导致了新型治疗方法的批准。在这篇综述中，我们提供了流行病学、发病机制、临床表现的概述，并回顾了诱导和维持缓解的治疗策略。
