| 摘要: |
| 目的 探讨膜性肾病（membranous nephropathy，MN）合并抗中性粒细胞胞浆抗体（anti-neutrophil cytoplasm antibodies，ANCA）相关性新月体肾炎的临床病理特点和预后情况。 方法 将检索全球中英文文献的79例报告和我院1例共计80例有完整临床病理资料的MN合并ANCA相关性新月体肾炎病例纳入本研究，总结此类疾病的临床资料、实验室检查结果、病理特点、治疗方案和预后情况，加强对该病的认识。结果 80例病例中男性44例，女性36例，平均年龄56.8±13.1岁，平均病程3.2±3.6月，95%（76/80）的病例MN与ANCA相关性新月体肾炎同时发生，93.8%（75/80）的患者起病时合并肾功能不全，最常见的肾脏表现为肾病综合征合并急进性肾炎。所有患者均存在血清ANCA阳性，88.2%（60/68）MPO-ANCA阳性，起病时24h尿蛋白定量5.27±4.3g，血肌酐420.7±307μmol/L。肾脏病理最常见的表现为新月体形成和基底膜增厚，免疫荧光以IgG和C3沿毛细血管袢颗粒样沉积为主。激素联合环磷酰胺（cyclophosphomide，CTX）治疗可显著的改善患者预后，治疗后62.7%（32/51）的病例可达到肾功能稳定或缓解。结论 膜性肾病合并ANCA相关性新月体肾炎者较为罕见，两者发病机理的相关性尚不明确，积极的免疫抑制治疗可改善预后。 |
| 关键词: 膜性肾病 抗中性白细胞胞质抗体 新月体肾炎 血管炎 活组织检查 |
| DOI：10.16781/j.0258-879x.2016.11.1440 |
| 投稿时间：2016-07-05修订日期：2016-08-31 |
| 基金项目:上海市卫生局青年科研项目（2012106），第二军医大学长海医院“1255”学科建设计划项目（CH125540100）. |
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| Membranous glomerulonephritis combined with anti-neutrophil cytoplasm antibody-associated crescentic glomerulonephritis |
| BIAN Qi,CHEN Zhou,GUO Zhi-yong,CUI Ruo-lan,YU Guang* |
(Department of Nephrology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China
*Corresponding author.) |
| Abstract: |
| Objective To study the clinicopathological features of membranous
nephropathy(MN) with ANCA-associated crescentic glomerulonephritis (ANCA-associated CGN). Methods 79 cases diagnosed as MN with ANCA associated CGN were selected from the whole English and Chinese literatures and a similar case was from our hospital. Total 80 casess were included in this study to summarize the clinicopathological features, treatment and prognosis. Results 44 male and 36 female patients were included. The average age was 56.8±13.1 years and the average disease onset period was 3.2±3.6 months. In 95% cases, MN and ANCA associated CGN occurred simultaneously. 93.8% patients presented renal dysfunction onset of the disease, the common clinical manifestation were nephrotic syndrome with rapidly progressive glomerulonephritis. All patients were serum ANCA positive and 88.2% cases were MPO-ANCA positive. The average 24h proteinuria was 5.27±4.3g and SCr was 420.7±307μmol/L. Renal biopsy showed crescent formation and GBM thicken. Immunofluorescence showed IgG and C3 deposits were positive. Prednisone combined with CTX could improve the prognosis. 62.7% cases reached relieve remission. Conclusions The coexistence of ANCA associated CGN and MN was rare. The pathogenesis of this condition is still unclear. Immunosuppressive therapy might improve the outcome. |
| Key words: membranous nephropathy antineutrophil cytoplasmic antibodies crescentic glomerulonephritis vasculitis bioposy |
