戴彧君　陈志祥　张弛　佘艳军

[摘要] 抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是一种累及多个系统且呈坏死性的血管炎。该病所涉及的系统比较零散，典型症状不明显，因此十分容易引起误诊、漏诊。且如果不进行及时有效的治疗，该病十分容易发展为终末期肾病，对患者的生命健康造成严重影响。因此，对该病进行早期诊断并进行及时合理治疗，控制病情的发展，可使患者预后得到明显改善。临床上常采用糖皮质激素联合细胞毒性药物作为AAV首选治疗方法。对于需要透析或血肌酐迅速升高、合并弥漫性肺出血、AAV和抗GBM肾炎重叠综合征的患者，建议加用血浆置换。由于AAV临床表现复杂，且缺少特异性实验室检查。因此临床医护人员需提高对该疾病的认识，才能及时准确地诊断并制定治疗方案。本文就1例AAV累及肾脏且需要透析者进行详细报道。

[关键词] ANCA;相关性血管炎;终末期肾病;透析

[中图分类号] R593.2          [文献标识码] C          [文章编号] 1673-9701（2021）06-0145-03

Clinical analysis of a dialysis patient with anti-neutrophil cytoplasmic antibody-associated vasculitis

Dai Yujun   CHEN Zhixiang   ZHANG Chi   SHE Yanjun

Departments of Nephrology， Southern Central Hospital of Yunnan Province， Mengzi   661100， China.

[Abstract] Anti-neutrophil cytoplasmic antibody（ANCA）-associated vasculitis （AAV） is a kind of necrotizing vasculitis involving multiple systems. The system involved in the disease is scattered and the typical symptoms are not obvious， so the misdiagnosis and missed diagnosis are frequently occurred. Moreover， without timely and effective treatment， the disease will easily develop into advanced stage， which will seriously affect the life and health of patients. Therefore， early diagnosis and timely and reasonable treatment can control the development of the disease and improve the prognosis of patients. Glucocorticoid combined with cytotoxic drugs is often used as the firs-line treatment for AAV. Plasma exchange is generally recommended for patients who need dialysis or whose serum creatinine rises rapidly， complicated with diffuse pulmonary hemorrhage， AAV and anti-GBM nephritis overlap syndrome. Due to the fact that the clinical manifestations of AAV are complex and lack of specific laboratory tests， medical staff need to improve their understanding of the disease， so that they can diagnose the disease timely and accurately and make treatment plans. A case of AAV involving kidney and requiring dialysis was reported in detail.

[Key words] ANCA; Associated vasculitis; End-stage renal disease; Dialysis

抗中性粒细胞胞浆抗体（Anti-neutrophil cytoplasmic antibody，ANCA）相关性血管炎（Associated Vasculitis，AAV）是一种系统性、坏死性血管炎，属自身免疫性疾病，多发于中老年人群。由于其侵犯全身多个器官，进而导致相应的临床症状[1]，极易累及肺部及肾脏。调查显示，该病累及肾脏者有80%之多[2]，且肾脏被认为是AAV损害的最常见靶器官，如不及时治疗，则会使病情快速恶化，对患者的生命安全造成严重威胁。调查显示，1年内未经过治疗的患者死亡率高达80%[3]，其治病机制是由于ANCA在诱导激活后，其补体进一步活化所引发的炎症反应，使小血管壁发生炎症反应就纤维素样坏死[4]，研究指出，蛋白酶3（Protease 3，PR3）与髓过氧化物酶（Myeloperoxidase，MPO）是其靶抗原[5]。……