Molnár Adél, Studinger Péter, Ledó Nóra
Department of Internal Medicine and Oncology, Semmelweis University, Budapest, Hungary.
Front Med (Lausanne). 2022 Jun 3;9:884188. doi: 10.3389/fmed.2022.884188. eCollection 2022.
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a destructive small vessel vasculitis affecting multiple organs. Renal involvement often leads to end-stage renal disease and increases mortality. Prompt diagnosis and initiation of adequate immunosuppressive therapy are critical for the best patient and kidney outcomes. However, considerable heterogeneity in symptoms and severity across the patients frequently hinder the diagnosis and management. The objective of this review is to emphasize the heterogeneity of the ANCA-associated vasculitis, facilitate the recognition and give guidance to the therapeutical possibilities. We present epidemiologic and risk factors, pathogenesis, and provide comprehensive clinical features of the disease. This article also focuses on the currently available therapeutic options and emerging cellular and molecular targets for the management of systemic and especially renal disease. We conducted extensive literature research published on PubMed and Google Scholar. We systematically reviewed, analyzed, and assembled databases, covering a broad spectrum of aspects of the disease. We compared and summarized the recommendations of two recent guidelines on ANCA-associated vasculitis. The incidence of ANCA-associated vasculitis, hence glomerulonephritis shows a steady increase. Familiarity with the presenting symptoms and laboratory abnormalities are necessary for rapid diagnosis. Early initiation of treatment is the key aspect for favorable patient and renal outcomes. A better understanding of the pathogenesis constantly leads to more targeted and therefore more efficient and less toxic treatment.
抗中性粒细胞胞浆自身抗体（ANCA）相关血管炎是一种累及多个器官的破坏性小血管炎。肾脏受累常导致终末期肾病并增加死亡率。及时诊断并开始适当的免疫抑制治疗对于患者和肾脏的最佳预后至关重要。然而，患者症状和严重程度的显著异质性常常阻碍诊断和管理。本综述的目的是强调ANCA相关血管炎的异质性，促进识别并为治疗可能性提供指导。我们介绍了流行病学和危险因素、发病机制，并提供了该疾病的全面临床特征。本文还重点关注了目前可用的治疗选择以及用于治疗全身性尤其是肾脏疾病的新兴细胞和分子靶点。我们对发表在PubMed和谷歌学术上的文献进行了广泛研究。我们系统地回顾、分析并整合了数据库，涵盖了该疾病的广泛方面。我们比较并总结了最近两份关于ANCA相关血管炎指南的建议。ANCA相关血管炎以及肾小球肾炎的发病率呈稳步上升趋势。熟悉呈现出的症状和实验室异常情况对于快速诊断是必要的。早期开始治疗是实现良好患者和肾脏预后的关键方面。对发病机制的更好理解不断带来更具针对性、因此更有效且毒性更小的治疗方法。
