anca相关性血管炎的病理生理学。

Q4 Medicine
Ceskoslovenska patologie Pub Date : 2020-01-01
Bartoňová Lenka, Hrušková Zdenka, Honsová Eva
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引用次数: 0

摘要

ANCA阳性血管炎(AAV)是一种严重的自身免疫性疾病,主要影响各器官系统的小血管,伴有血清中ANCA抗体的存在。AAV是一组成人中最常见的系统性血管炎,根据临床表现,该疾病实体包括3种表型,即:肉芽肿合并多血管炎(原Wegeners肉芽肿病)、显微镜下的多血管炎和嗜酸性肉芽肿合并多血管炎(原Churg-Strauss综合征)。与其他自身免疫性疾病类似,AAV发生在暴露于致病环境因素(如感染)的易感遗传背景的患者中。ANCA抗体引起血管炎的机制涉及过度的中性粒细胞活化,随后导致释放促炎细胞因子、活性氧和裂解酶。此外,活化的中性粒细胞在称为NETosis的过程中诱导中性粒细胞胞外陷阱的形成。释放的中性粒细胞抗原通过抗原呈递细胞暴露于免疫系统,进一步刺激抗体的产生,造成组织破坏的恶性循环。了解AAV的发病机制不仅是提供最佳诊断和治疗的关键,而且还为病理学家更深入地了解疾病进展的形态学特征,包括发展和愈合的各个阶段提供了工具。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pathophysiology of ANCA-associated vasculitis.

ANCA positive vasculitis (AAV) is a serious autoimmune disease mainly affecting small vessels in various organ systems, accompanied by the presence of ANCA antibodies in serum. AAV represents a group of the most common systemic vasculitis in adulthood, and based on clinical manifestations this disease entity includes 3 phenotypes, namely: granulomatosis with polyangiitis (formerly Wegeners granulomatosis), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Similar to other autoimmune diseases, AAV develops in patients with a predisposing genetic background who have been exposed to causative environmental factors, such as infections. The mechanisms by which ANCA antibodies cause vasculitis involves excessive neutrophil activation, that subsequently leads to release pro-inflammatory cytokines, reactive oxygen species and lytic enzymes. In addition, activated neutrophils induce the formation of neutrophil extracellular traps in a process called NETosis. The released neutrophil antigens are exposed to the immune system via antigen presenting cells, which further stimulates antibody production and creates a vicious circle with tissue destruction. Understanding the pathogenesis of AAV represents the key which provides not only optimal diagnosis and treatment, but also gives the pathologist a tool for deeper insight into the morphological features of disease progression, including the various stages of development and healing.

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来源期刊
Ceskoslovenska patologie
Ceskoslovenska patologie Medicine-Medicine (all)
CiteScore
0.40
自引率
0.00%
发文量
17
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