本文由小咖机器人翻译整理

期刊来源：Lancet

原文链接：https://doi.org/10.1016/S0140-6736(23)01736-1

摘要内容如下：

抗中性粒细胞胞浆抗体（ANCA）相关性血管炎包括两种主要疾病，肉芽肿病伴多血管炎和显微镜下多血管炎，并且仍然是最具破坏性和潜在致命性的自身免疫性炎症性疾病之一。肉芽肿病伴多血管炎和显微镜下多血管炎的特征是坏死性血管炎，几乎可累及任何器官，通常一起进行研究。这些疾病通常影响肾、肺、上呼吸器官、皮肤、眼睛和周围神经。肉芽肿性炎和多核巨细胞是肉芽肿性多血管炎的主要病理特征，但在显微镜下多血管炎中不存在。许多免疫系统事件对于疾病的发病机理是必需的，例如补体旁路的激活、通过补体受体的中性粒细胞激活以及炎性细胞（包括单核细胞和巨噬细胞）的流入。这些细胞使炎症持续存在并导致器官损伤。在21世纪，ANCA相关血管炎的治疗已经从依赖细胞毒性药物转向诱导和维持疾病缓解的靶向生物药物。更早的诊断，部分是更可靠的ANCA检测的结果，改善了患者的预后和生存率。急性疾病相关死亡率的降低现已转移到与ANCA相关血管炎及其治疗相关的长期发病率，如慢性肾病和心血管疾病。临床试验和临床实践中的治疗方法仍然过于依赖糖皮质激素，继续努力降低糖皮质激素的毒性仍然是开发新治疗策略的优先事项。

英文原文如下：

Abstracts

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis consists of two main diseases, granulomatosis with polyangiitis and microscopic polyangiitis, and remains among the most devastating and potentially lethal forms of autoimmune inflammatory disease. Granulomatosis with polyangiitis and microscopic polyangiitis are characterised by a necrotising vasculitis that can involve almost any organ, and have generally been studied together. The diseases commonly affect the kidneys, lungs, upper respiratory tract, skin, eyes, and peripheral nerves. Granulomatous inflammation and multinucleated giant cells are key pathological hallmarks of granulomatosis with polyangiitis, but are absent in microscopic polyangiitis. Many immune system events are essential to disease aetiopathogenesis, such as activation of the alternative complement pathway, neutrophil activation via complement receptors, and the influx of inflammatory cells, including monocytes and macrophages. These cells perpetuate inflammation and lead to organ damage. During the 21st century, the management of ANCA-associated vasculitis has moved away from reliance on cytotoxic medications and towards targeted biological medications for both the induction and maintenance of disease remission. Earlier diagnosis, partly the result of more reliable ANCA testing, has led to improved patient outcomes and better survival. Reductions in acute disease-related mortality have now shifted focus to long-term morbidities related to ANCA-associated vasculitis and their treatments, such as chronic kidney disease and cardiovascular disease. Therapeutic approaches in both clinical trials and clinical practice still remain too reliant on glucocorticoids, and continued efforts to reduce toxicity from glucocorticoids remain a priority in the development of new treatment strategies.

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