韦格纳肉芽肿病临床分析  

Wegener＇s granulomatosis clinical analysis

作　　者：胡云衢[1] 任海波[2] 孙世安[3] 廖娟[1] 李玉柱[4] 

机构地区：[1]武警新疆总队医院呼吸科,乌鲁木齐830091 [2]武警新疆总队医院麻醉科,乌鲁木齐830091 [3]武警新疆总队医院门诊部,乌鲁木齐830091 [4]解放军总医院呼吸科,北京100853

出　　处：《国际呼吸杂志》2013年第9期680-683,共4页International Journal of Respiration

摘　　要：目的探讨韦格纳肉芽肿病的临床特点，以提高对本病的认识。方法回顾性分析1992年4月至2012年5月确诊的50例韦格纳肉芽肿病患者的临床资料，包括临床表现，实验室、影像学及病理检查等。结果男22例，女28例，发病年龄为13～74岁，平均43．8岁。确诊时间为14d～180个月，平均16．7个月。临床以眼（38％）、耳（20％）、鼻（64％）、口咽（26％）等上呼吸道及肺部（58％）和肾（44％）受累为主。胞质型抗中性粒细胞胞质抗体（cANCA）阳性率96％，核周型抗中性粒细胞胞质抗体（pANCA）阳性率12％。病理表现为坏死性肉芽肿性炎症、炎细胞浸润的血管炎。治疗以糖皮质激素和免疫抑制剂为主。结论韦格纳肉芽肿病的临床表现复杂多样，缺乏特异性体征，靶器官受损有多系统或器官病变的症候群，尤以上、下呼吸道及肾脏受累最多见，病理特点为坏死性肉芽肿和血管炎。Objective Explore the clinical features of Wegener＇ s granulomatosis, in order to improve the understanding of the disease. Methods Retrospective analysis of 50 cases diagnosed in April 1992 to May 2012, the clinical data of patients with Wegener＇ s granulomatosis, including clinical manifestations, laboratory, radiological and pathological examination. Results 22 males,28 females, age of onset is 13-74 years old, with an average age of 43.8 years. Diagnosis time for 14 days 180 months, an average of 16. 7 months. Clinical eyes （38%）, ear （20~A）, nose （64%）, oropharynx （26%）, upper respiratory tract and lungs （58%） and renal （44%） is mainly involved. Cytoplasmic anti-neutrophil cytoplasmic antibodies （cANCA） positive rate of 96 ~, perinuclear anti-neutrophil cytoplasmic antibodies （pANCA）-positive rate of 12 %. Pathological manifestations of necrotizing granulomatous inflammation, vasculitis, inflammatory cell infiltration. Treatment mainly glucocorticoids and immunosuppressants. Conclusions Complex and diverse clinical manifestations of Wegener＇s granulomatosis, lack of specific signs, target organ damage how the system or organ disease syndrome, especially over the most common lower respiratory tract and renal involvement, the pathological features of necrotizing granulomatous vasculitis.

关 键 词：韦格纳肉芽肿 多系统表现 临床分析 

分 类 号：R593.9[医药卫生—内科学]