抗中性粒细胞胞浆抗体相关性血管炎
ANCA-associated vasculitis.
作者信息
Yates Max, Watts Richard
机构信息
Norfolk and Norwich University Hospital NHS Foundation Trust, and Norwich Medical School, University of East Anglia, Norwich, UK.
Ipswich Hospital and honorary senior lecturer Norwich Medical School, University of East Anglia, Norwich, UK.
出版信息
Clin Med (Lond). 2017 Feb;17(1):60-64. doi: 10.7861/clinmedicine.17-1-60.
Abstract
The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome). This review examines recent developments in the pathogenesis and treatment of AAV, focusing on developments in treatment following the introduction of rituximab, in particular.
摘要
血管炎是一组异质性疾病，其典型特征是能够引起血管炎症，可伴有或不伴有坏死。它们表现出各种各样的体征和症状，如果不进行治疗，会带来严重的死亡率和发病率负担。抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）包括三种不同的疾病——肉芽肿性多血管炎（GPA；以前称为韦格纳肉芽肿）、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎（EGPA；以前称为变应性肉芽肿性血管炎）。本综述探讨了AAV发病机制和治疗方面的最新进展，尤其关注利妥昔单抗引入后治疗方面的进展。
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