肉芽肿性多血管炎合并急性肾功能衰竭临床及病理分析  

Clinicopathological analysis of patients with granulomatosis with polyangiitis （Wegener＇s） complicated by acute renal failure

作　　者：杨波[1] 沈敏[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院风湿免疫科,100032

出　　处：《中华医学杂志》2013年第15期1159-1161,共3页National Medical Journal of China

基　　金：基金项目：北京协和医院青年科研基金（1605300）

摘　　要：目的分析肉芽肿性多血管炎（GPA）并发急性肾功能衰竭（ARF）的临床及病理特点，提高对此危重症的认识。方法回顾性分析北京协和医院1988至2011年收治的共27例GPA合并ARF患者，其临床表现、实验室检查、肾脏病理特点、治疗和转归。结果GPA合并ARF的发生率约15％（27／180），27例患者发病平均年龄（50±13）岁。全身非特异性症状以发热（23例）、体重减轻（15例）多见，肾外表现以肺（20例）、鼻（19例）受累多见。肾脏表现除均有血肌酐急剧升高外，还包括少到中量蛋白尿（26例）和血尿（25例），少尿或无尿罕见。23例患者cANCA阳性，多数（22例）识别PR3。典型肾脏病理表现为寡免疫复合物局灶节段坏死型肾小球肾炎或新月体肾炎。应用激素及免疫抑制剂治疗后病情缓解者22例，4例患者死亡。结论GPA合并ARF临床并不少见，其起病急、进展快，少尿及无尿罕见。对于以ARF为首发表现的患者应注意检测ANCA。肾脏病理对于疾病诊断、病情判定、治疗决策和预后判断具有重要意义。Objective To analyze the elinieopathological features of patients with granulomatosis with polyangiitis （Wegener＇s） （GPA） complicated by acute renal failure （ARF）. Methods A total of 27 GPA patients with ARF at our hospital from January 1988 to December 2011 were recruited into this retrospective study. A structured interview, systemic rheumatologic examination, laboratory tests （including autoantibodies） and renal pathology were conducted. And their treatment and prognosis were also analyzed. Results The incidence of GPA with ARF was 15% and the average age 50 years old. The common symptoms were fever （n =23） and weight loss （n = 15） while the most common extra-renal organ damages affected lung （n = 20） and nose （n = 19）. Renal damages manifested as mild or moderate proteinuria （n = 26） and hematuria （n = 25 ）. However, oliguria and anuria were rare. Anti-neutrophil cytoplasma antibodies （ANCA） were positive in 23 patients. Among them, 22 were identified by proteinase 3. Renal biopsies revealed pauci-immune crescentic glomerulonephritis. Combined treatment of glucocorticoid and immunosuppressants was effective. Twenty-two patients became relieved and only 4 patients died. Conclusion As a common complication of GPA, ARF has a rapid onset and a fast progression. Oliguria and anuria are rare. Renal biopsy is not only vital in the diagnosis and evaluation of disease activity, but also helpful for therapeutic decision-making.

关 键 词：肉芽肿 多血管炎 急性肾功能衰竭 肾小球肾炎 

分 类 号：R593[医药卫生—内科学] R692[医药卫生—临床医学]