抗中性粒细胞胞浆抗体相关性小血管炎肾损害病例报告并文献复习

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1、授予单位代码10089学号或申请号20153331中国图书分类号R692HebeiMedicalUniversity硕士学位论文专业学位抗中性粒细胞胞浆抗体相关性小血管炎肾损害病例报告并文献复习研究生：郭宝珠导师：李英教授专业：内科学二级学院：第三医院2018年3月目录中文摘要·················································································1英文摘要·····································

2、············································2英文缩写·················································································4研究论文抗中性粒细胞胞浆抗体相关性小血管炎肾损害病例报告并文献复习前言·················································································5病例简介··········

3、·································································6附图················································································11附表···············································································14讨论···························

4、····················································15结论···············································································21参考文献·········································································21综述溶酶体相关膜蛋白-2及其自身抗体在AASV肾损害中的研究进展··············

5、····································································30致谢·····················································································37个人简历···············································································38中文摘要抗中性粒细胞胞浆抗体相关性小

6、血管炎肾损害病例报告并文献复习摘要目的：抗中性粒细胞胞浆抗体（Antineutrophilcytoplasmicantibody，ANCA）相关性血管炎（ANCA-associatedsystemicvascufitis，AASV）是一种自身免疫性疾病。通过对其流行病学的研究发现，发病率呈上升趋势。该疾病临床表现多样，常为多系统受累，以肾脏、肺受累最常见，但缺乏特异性症状，导致漏诊及误诊率极高，治疗干预不及时，预后较差。通过本文病例报道及相关文献复习，充分认识其早期临床表现及辅助检查的异常，明确诊断并制定有效的治疗方案

7、，为今后该疾病的诊疗提供临床指导。方法：通过研究我科收治的1例以间断发热及双下肢水肿为主要首发症状的AASV肾损害患者，总结其病例特点，对其病程进展，诊疗过程、相关的检查化验及病理结果做一报道，并查阅相关文献，提高对该疾病发病机制、临床特征和治疗方案的了解掌握，降低误诊及漏诊率，尽早干预，改善患者预后。结果：患者男性，51岁，主因间断性发热40余天，双下肢水肿3天于我院就诊，结合其临床表现及辅助检查，并经肾穿刺活检证实为血管炎肾损害病理表现，给予糖皮质激素联合环磷酰胺及抗感染等治疗后，症状改善，复查血清学ANCA较前下

8、降，肾功能受损未再进展，病情缓解出院。结论：AASV的早期诊断及早期治疗至关重要，若未及时诊断和干预，其死亡率较高，尤其累及肾脏时，一般表现为寡免疫坏死性新月体性肾小球肾炎，进展较快，预后不佳。当出现镜下血尿、水肿及其他全身多系统受累表现时应考虑此病，及时进行血清学ANCA检测及肾穿刺活检术，明确诊断，制定治疗方案。关键词：AAS