韦格纳肉芽肿病的临床病理表现  

Clinical and pathological manifestations of patients with Wegener's granulomatosis

作　　者：赵明辉[1] 刘娜[1] 章友康[1] 邹万忠[1] 刘玉春[1] 姜筠[1] 王海燕[1] 

机构地区：[1]北京医科大学第一医院肾内科

出　　处：《北京医科大学学报》1999年第4期328-330,共3页Journal of Peking University(Health Sciences)

摘　　要：目的：认识韦格纳肉芽肿病的临床病理表现。方法：对北京医科大学第一医院肾内科近３年来经病理检查确诊的６例韦格纳肉芽肿病病例进行临床病理资料的分析和总结。患者血清均行抗中性粒细胞胞浆抗体（ａｎｔｉｎｅｕｔｒｏｐｈｉｌｃｙｔｏｐｌａｓｍｉｃａｕｔｏａｎｔｉｂｏｄｉｅｓ，ＡＮＣＡ）检测，ＩＩＦ法（ｉｎｄｉｒｅｃｔｉｍｍｕｎｏｆｌｕｏｒｅｓｃｅｎｃｅ）区分ｃＡＮＣＡ和ｐＡＮＣＡ，抗原特异性ＥＬＩＳＡ以高度纯化的ＰＲ３（ｐｒｏｔｅｉｎａｓｅ３）和ＭＰＯ（ｍｙｅｌｏｐｅｒｏｘｉｄａｓｅ）为靶抗原。结果：６例患者男性２例，女性４例，平均年龄５６．８（２６～６８）岁；ｐＡＮＣＡ／ＭＰＯＡＮＣＡ阳性４例，ｃＡＮＣＡ／ＰＲ３ＡＮＣＡ阳性２例；６例均经活检病理检查发现肉芽肿性病变，肉芽肿中心多为纤维素样坏死性小血管炎，周围炎性细胞浸润及类上皮样细胞增生，其中４例发现于肾间质，１例于肺结节和１例于鼻粘膜；临床表现为多器官受累，均有肾受累，肾活检除可见肉芽肿性小血管炎外，肾小球均可见毛细血管壁局灶节段性纤维素样坏死伴或不伴新月体形成；所有患者均有肺受累，表现为咳嗽，咯血，胸片可见肺部阴影、结节或空洞；其他器官受累也较常见，包括眼、?Objective: To analyze the clinical and pathological manifestations of patients with Wegener's granulomatosis. Methods: Clinical and pathological data were collected from six patients with pathologically confirmed Wegener's granulomatosis and were further analyzed. Antineutrophil cytoplasmic autoantibodies (ANCA) were detected in all sera, and standard IIF (indirect immunofluorescence) method and antigen specific ELISA, using PR3 (proteinase 3) and MPO (myeloperoxidase) as solid phase ligands, were employed. Results: Of the six patients, four were female and two male with an average age of 56.8 (26-68) years old. Four patients were pANCA/MPO ANCA positive and two cANCA/PR3 ANCA positive. All six patients were found to have vasculitic granuloma in their biopsy tissues. Four were from renal interstitium, one from lung nodule and one from nosal mucosa. Clinically, all patients had multi system involvement, especially kidney and lungs. All patients had hematuria and proteinuria, and renal biopsies showed focal segmental necrotizing glomerulonephritis with crescents formation except for granulomatous vasculitis. Patients with lung involvement had cough and cough blood. Chest X ray showed pulmonary shadows, nodules or cavities. Eye, ear, skin, joints, muscle and peripheral nerve systems were also frequently involved. In laboratory examinations, all patients had anemia with elevated ESR and CRP. WBC and platelet counts were increased. All six patients had fever and fatigue, four of whom had body weight loss. An early diagnosis and appropriate treatment suggested a better prognosis, otherwise, the patients could reach end stage renal failure progressively. Conclusion: Wegener's granulomatosis is an ANCA related small vessel vasculitis. In this study, the target ANCA antigens were MPO and PR3. The patients were middle to old aged. They all had multi system involvement clinically and granulomatous vasculitis in pathology. For patients with suspected small vessel vasculitis, an ANCA test should be performed in order

关 键 词：韦氏肉芽肿 病理学 诊断 

分 类 号：R593.902[医药卫生—内科学]