ANCA相关性血管炎肾损害合并交通性脑积水一例并文献复习  

Antineutrophil cytoplasmic antibody-associated vasculitis with renal involvement complicated with communicating hydrocephalus:a case report and literature review

作　　者：蒋兰萍[1] 杨诗聪[2] 王欣[1] 许娴[1] 文琼[1] 李志坚[1] 陈雄辉[1] Jiang Lanping;Yang Shicong;Wang Xin;Xu Xian;Wen Qiong;Li Zhijian;Chen Xionghui(Department of Nephrology,the First Affiliated Hospital of Sun Yat-sen University,Key Laboratory of Nephrology,National Health Commission of China and Guangdong Province,Guangzhou 510080,China)

机构地区：[1]中山大学附属第一医院肾内科,国家卫生健康委员会重点实验室,广东省肾脏病重点实验室,广州510080 [2]中山大学附属第一医院病理科,广州510080

出　　处：《新医学》2021年第11期873-878,共6页Journal of New Medicine

基　　金：国家自然科学基金(81600545);国家重点研发计划(2017YFC0907601,2017YFC0907602,2017YFC0907603,2016YFC0906100,2016YFC0906101);广东省肾脏病重点实验室(2020B1212060028);国家卫生健康委员会重点实验室;2021年中山大学重点发展项目研究生教育质量提升专项基金(80000-18842270)。

摘　　要：目的探讨合并交通性脑积水的ANCA相关性血管炎(AAV)的发病机制、诊治方法,提高临床医师对AAV罕见类型的认识水平。方法收集1例以交通性脑积水为首发症状的AAV病例的临床资料,以"ANCA相关性血管炎""脑积水"为关键词(包括中英文)对PubMed和CNKI收录的论文进行检索,结合文献分析病例特点、实验室检查、影像学检查、肾脏活组织检查(活检)和诊治方法。结果该例71岁男性患者,因头晕伴行走不稳半年余在门诊行CT检查提示交通性脑积水,拟行手术治疗。术前检查发现血清肌酐635μmol/L,核周型-抗中性粒细胞胞质抗体(p-ANCA)(+),MPO 293 U/L,尿红细胞位相示畸形红细胞240 000/μL,经肾脏活组织检查(活检)证实存在AAV肾损害。随访30个月,经糖皮质激素(激素)联合环磷酰胺治疗后,患者血清肌酐降至335μmol/L并脱离透析,头晕、行走不稳、智力减退症状逐渐好转,后多次复查颅脑MRI,交通性脑积水较前无明显变化。检索数据库后共收集5篇相关病例报道,患者年龄33~76岁,临床表现为脑积水和AAV,予激素联合免疫抑制剂、脑室-腹膜分流术等治疗,随访期间1例患者死亡,另外4例患者症状缓解。结论以脑积水起病的AAV罕见且血管炎症状隐匿,易被误诊或漏诊,对不明原因伴肾衰竭的神经系统受累病变进行ANCA筛查十分必要,肾穿刺活检有助于明确诊断,及时正规治疗可缓解脑积水症状,有助改善患者预后。Objective To investigate the pathogenesis, diagnosis and treatment of antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis complicated with communicating hydrocephalus and deepen the clinicians’ understanding of rare types of ANCA-associated vasculitis. Methods Clinical data of 1 case of ANCA-associated vasculitis presenting with the onset symptom of communicating hydrocephalus were collected. Literature review was conducted by using the keywords of “ANCA-associated vasculitis” and “hydrocephalus” from PubMed and China National Knowledge Infrastructure(CNKI) database. Clinical characteristics, laboratory and imaging examination, renal biopsy pathology, diagnosis and treatment of this case were analyzed combined with literature. Results A 71-year-old male patient was admitted to the outpatient clinic due to “dizziness accompanied with walking instability for more than half a year”. CT scan prompted the diagnosis of communicating hydrocephalus, and he was scheduled to undergo surgery. Preoperative examination detected that serum creatinine level was 635 μmol/L, p-ANCA(+), MPO 293 U/L.The density of abnormal red blood cells in the urine was 240 000/μL. Renal biopsy confirmed the diagnosis of ANCA-associated vasculitis with renal damage. During 30-month follow up, he was treated with glucocorticoid combined with cyclophosphamide. Subsequently, the serum creatinine level was declined to 335 μmol/L and hemodialysis was discontinued. The symptoms of dizziness, walking instability and mental retardation at the onset of the disease were gradually relieved. Re-examination of brain MRI showed no significant changes of the communicating hydrocephalus. Through literature review, 5 relevant case reports were retrieved, aged from 33 to 76 years old, with clinical manifestations of hydrocephalus and ANCA-associated vasculitis. These patients were treated with glucocorticoid combined with immunosuppressants and ventricular-peritoneal shunt, etc. During follow-up, 1 patient died and relevant symptoms of

关 键 词：抗中性粒细胞胞质抗体相关性血管炎 肾损害 交通性脑积水 新月体肾炎 

分 类 号：R593.2[医药卫生—内科学] R742.7[医药卫生—临床医学]