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1、眼部Wegener肉芽肿12例临床分析作者:赵燕燕,魏世辉,黄厚斌单位:100853)中国北京市,解放军总医院眼科【摘要】目的:研究韦格纳肉芽肿病(wegenergranulomatosis,WG)的眼部表现、实验室检查、病理及影像学特点,提高眼科医师对本病的认识。方法:分析44例WG病例,入选其中有眼部表现的12例患者,冋顾性研究其眼部临床农现、实验室指标、病理结果以及影像学特点。结果:发病平均年龄39岁。从发病到确诊的平均吋间为llmoo眼部的任何一个部位都可能受累,包括眼球、附属器及视路。其中眼前节及眼眶最常累及。83%患
2、者ANCA结果阳性。病理表现有实质组织坏死、肉芽肿、多核巨细胞、微脓肿和血管炎等。CT、MRI是忖前辅助诊断WG的有效方法。结论:WG是一种致死性疾病,早期诊断和治疗至关重要,对于以眼科首诊和仅有眼部表现的可疑病例,眼科医生要注意鉴别。【关键词】韦格纳肉芽肿病;抗屮性粒细胞胞浆抗体;病理学;CT;MRI;眼部表现Clinicalanalysisof12patientswithocularWegenergranulomatosisYanYanZhao,ShiHuiWei,HouBinHuangDepartmentofOphthal
3、mology,GeneralHospitalofChinesePLA,Beijing100853,ChinaCorrespondenceto:ShiHuiWei.DepartmentofOphthalmology,GeneralHospitalofChinesePLA,Beijing100853,China,weishihui706@sohu.comAbstractAIM:Tostudytheoculai*features,laboratoryexaminations,imageologicalandpathologicalfe
4、aturesofWegenergranulomatosis(WG),soastoimprovemanagementofthisdisordenMETHODS:Aretrospectivestudyof12patientswithophthalmicinvolvementduetoWGwascarriedout.RESULTS:FortyfourpatientswerediagnosedtohaveWGOfthese,12hadophthalmicinvolvements・Thepatients′averageagea
5、tthebeginningofthediseasewas39years.Theaveragecoursefromtheinitiationofclinicalsymptomstodiagnosiswas11months・Anypartoftheeyeballcouldbeinvolvedin,butanteriorsegmentandorbitweremorecommon.Cytoplasmicpatternantineutrophilcytoplasmicantibodies(cANCA)werepositivein83%of
6、patients.Pathologicalmanifestationsinvolvednecrosis,granulomas,multinucleatedgiantcells,microabscesses,vasculitisandsoforth・CTandMRIwereeffectivemethodstodiagnoseWGespeciallyocularWGCONCLUSION:WGisanimportantclinicalentitythatneedstoberecognizedearlyandtreatedappropr
7、iately.Ophthalmicmanifestationsarefrequentlyencounteredandcanresultinsignificantmorbidityandevenblindness・CombinedassaysofANCAandtissuebiopsiesareneededforthediagnosisofWG.KEYWORDS:Wegenergranulomatosis;antineutrophilcytoplasmicantibody;pathology;CT;MRI:ophthalmicman
8、ifestations韦格纳肉芽肿(wegenergranulomatosis,WG)是•原因未明的全身进行性血管性坏死性肉芽肿疾病。中小血管甚至大动脉坏死性肉芽肿性血管炎为其病理特征,临床表现多样,典型性WG(classicalwegenergranuloma