肺韦格纳肉芽肿临床诊断学特点及文献复习

Clinical features and literature review of wegener granulomatosis

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。

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.