Abstract:ObjectiveTo investigate the clinical diagnostics features of wegener granulomatosis.MethodsThe 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.ResultsCT 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 0.5cm~1.0cm.Laboratory tests showed line anti-M2 mitochondrial antibodies were positive,ultra-sensitivity C-reactive protein was 9.54mg/L,erythrocyte sedimentation rate was 18mm/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 1cm.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.ConclusionsWG 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.
[1]曾平,张国华,苟丽娟,等.韦格纳肉芽肿合并肺部感染的临床特征[J].中华临床免疫和变态反应杂志,2011,5(3):212-216.
[2]杨志寅.现代医学科学发展中的缺憾与思考[J/CD].中华诊断学电子杂志,2013,1(1):1-7.
[3]乔琳,王迁,冷晓梅,等.“韦格纳肉芽肿”重新命名[J].中华临床免疫和变态反应杂志,2013,7(2):99-102.
[4]Cotch MF,Hoffman GS,Yerg DE,et al.The epidemiology of Wegener granulomatosis.Estimates of the five-year period prevalence,annual mortality,and geographic disease distribution from population-based data sources[J].Arthritis Rheum,1996,39(1):87-92.
[5]张玉梅,李爱民,刘志宏,等.韦格纳肉芽肿病45例临床和病理分析[J].山西医药杂志,2011,11(40):1078-1080.
[6]万齐,赵康艳,李新春,等.肺韦格纳肉芽肿病CT征象分析[J].放射学实践,2013,11(28):1128-1131.
[7]Franssen C,Gans R,Kallenberg C,et al.Disease spectrum of patients with antineutrophil cytoplasmic autoantibodies of defined specificity:distinct differences between patients with anti-proteinase 3 and anti-myeloperoxidase autoantibodies[J].J Intern Med,1998,244(3):209-216.
[8]于飞,袁虎,陈雷,等.1例无明确病理诊断的韦格纳肉芽肿并文献复习[J].中华耳科学杂志,2009,7(4):308-310.
[9]Takwoingi YM,Dempster JH.Wegener′s granulomatosis:an analysis of 33 patients seen over a 10-year period[J].Clin Otolaryngol Allied Sci,2003,28(3):187-194.
[10]Ferri E,Armato E,Capuzzo P,et al.Early diagnosis of Wegener′s granulomatosis presenting with bilateral facial paralysis and bilateral serousotitismedia[J].Auris Nasus Larynx,2007,34(3):379-382.
[11]Takwoingi YM,Dempster JH.Wegener granulomatosis an analysis of 33 patients seen over a 10-year period[J].Clin Otolaryngl Allied Sci,2003,28(3):187-194.
[12]刘国保,黎蕾,方心华,等.不同类型的韦格纳肉芽肿病的影像学对比[J].中国实验诊断学,2012,16(6):1044-1046.
[13]Falk RJ,Gross WL,Guillevin L,et al.Granulomatosis,with polyangiitis(Wegener):an alternative name for wegener granulomatosis[J].Arthritis Rheum,2011,63(4):863-864.
[14]Rosen MJ.Dr.Friedrich wegener and the ACCP,revisited [J].Chest,2007,132(6):1723-1724.