CAJ | 학술논문

目的：探讨韦格纳肉芽肿的临床诊断学特征。方法回顾性分析1例经手术及病理学证实的肺韦格纳肉芽肿患者的临床、实验室检验及影像学特征并文献复习。结果肺韦格纳肉芽肿患者胸部 CT 检查：双肺可见多个大小不等的类圆形密度增高影，边界清晰，有空洞形成，内外壁光滑。纵隔内可见多个直径约0．5 cm ～1．0 cm 的淋巴结影。实验室检查所见：抗 M2型线粒体抗体阳性，超敏 C 反应蛋白9．54 mg／L，红细胞沉降率18 mm／h；抗核抗体阴性，蛋白芯片各项肿瘤标志物结果正常，结核实验阴性，真菌 D 葡聚糖检测阴性，抗中性粒细胞胞浆抗体胞浆型、抗中性粒细胞胞浆抗体核周型、抗髓过氧化物酶抗体、抗蛋白酶3抗体、抗肾小球基膜均阴性。病变术中直视下所见：右肺上叶可见数个大小不等结节，最大直径约1 cm。所取活检组织切面呈灰白色，病变镜下印象：肺组织内血管内皮增生，管壁增厚，多量嗜酸性粒细胞浸润伴周围大量组织细胞反应。结论韦格纳肉芽肿是一种累及呼吸道、肺、肾的坏死性肉芽肿性血管炎，发病率低，预后较差；及时明确诊断是治疗关键，在影像学及实验室检查尚不能明确诊断的情况下，可选择腔镜下活检。
목적：탐토위격납육아종적림상진단학특정。방법회고성분석1례경수술급병이학증실적폐위격납육아종환자적림상、실험실검험급영상학특정병문헌복습。결과폐위격납육아종환자흉부 CT 검사：쌍폐가견다개대소불등적류원형밀도증고영，변계청석，유공동형성，내외벽광활。종격내가견다개직경약0．5 cm ～1．0 cm 적림파결영。실험실검사소견：항 M2형선립체항체양성，초민 C 반응단백9．54 mg／L，홍세포침강솔18 mm／h；항핵항체음성，단백심편각항종류표지물결과정상，결핵실험음성，진균 D 포취당검측음성，항중성립세포포장항체포장형、항중성립세포포장항체핵주형、항수과양화물매항체、항단백매3항체、항신소구기막균음성。병변술중직시하소견：우폐상협가견수개대소불등결절，최대직경약1 cm。소취활검조직절면정회백색，병변경하인상：폐조직내혈관내피증생，관벽증후，다량기산성립세포침윤반주위대량조직세포반응。결론위격납육아종시일충루급호흡도、폐、신적배사성육아종성혈관염，발병솔저，예후교차；급시명학진단시치료관건，재영상학급실험실검사상불능명학진단적정황하，가선택강경하활검。
Objective To investigate the clinical diagnostics features of wegener granulomatosis. Methods The clinical,ultrasonographic,serum biochemical and computed tomography(CT)data of wegener granulomatosis (WG)confirmed by surgery and pathology was retrospectwely analyzed,and relevant literature were reviewed.Results CT examination showed there were multiple nodules and cavitations of lung,the boundary was clear,and the diameter of the mediastinal lymph node shadow was about 0.5 cm ~1 .0 cm. Laboratory tests showed line anti-M2 mitochondrial antibodies were positive,ultra-sensitivity C-reactive protein was 9.54 mg/L,erythrocyte sedimentation rate was 1 8 mm/H,antinuclear antibody was negative, various tumor marker detected by protein chip were normal;tuberculosis test was negative;fungi D glucan was negative;cANCA,pANCA,MPO,PR3,GBM were negative.The direct version of lesions during operation showed a number of different sizes nodules in right upper lobe and the largest diameter was about 1 cm. Section surface of the nodule was gray.The microscopic version of lesions showed the lung tissue vascular endothelial was proliferated,wall was thickening,eosinophil infiltration with histiocyte reaction around. Conclusions WG is a rare granulomatous necrotizing vasculitis involving upper and lower respiratory tracts and the kidneys.Timely diagnosis is the key to the treatment.WG can be diagnosed with endoscopic biopsy in case of unclear radiological and laboratory diagnosis.