韦格纳肉芽肿病100例临床分析  

Clinical analysis of 100 patients with Wegener＇s granulomatosis

作　　者：张国华[1] 吴庆军[1] 张立民[1] 曾小峰[1] 

机构地区：[1]中国医学科学院北京协和医院风湿免疫科,100032

出　　处：《中华风湿病学杂志》2010年第10期677-681,共5页Chinese Journal of Rheumatology

摘　　要：目的 总结分析100例韦格纳肉芽肿病（WG）患者的临床持点方法回顾性分析北京协和医院1998年1月至2009年12月确诊的100例WG患者的临床资料结果100例患者的男女比例1.04∶1,发病年龄4～72岁,平均（39±17）岁.上呼吸道（86%）、肺脏（82%）、肾脏（70%）和眼（53%）受累常见,神经系统（12%）及心脏（11%）等相对少见.胞质型抗中性粒细胞胞质抗体（cANCA）阳性率为77%,ANCA阴性率为8%.影像学以肺部多发结节/肿块影伴或不伴空洞病灶（59%,48/81）及鼻窦炎（57%,44/77）最常见.具有诊断意义的病理为坏死性肉芽肿和（或）小血管炎,鼻黏膜/肿中物及肺活检分别占78%及75%,肾活检以局灶节段坏死性肾小球肾炎（59%）最有诊断意义.WG亚组以全身型（49%）最常见,其次为局限型（22%）、早期全身型（15%）、重症肾脏病型（9%）及难治型（5%）.激素联合环磷酰胺治疗,诱导缓解期总缓解率为78%,其中早期全身型最高（73%）,病死率为4%.随访1～145个月,16例（22%）合并感染,9例（12%）合并慢性肾功能不全,8例（11%）并发深静脉血栓5例（8%）死亡,其中2例死于感染结论WG临床表现复杂多样,综合ANCA、影像学和组织活检利于早期诊断,早期治疗,减少并发症的发生,改善预后.Objective To investigate the clinical features of 100 cases with Wegener＇s granulomatosis （WG）. Methods One hundred patients with WG admitted to our center in recent 11 years were retrospectively analyzed. Results The ratio of male to female was 1.04：1. The average age was （39±17） years （ranging from 4 to 72 years）. The upper respiratory tract （86%）, lung （82%）, kidney （70%） and ocular （53%） were the major affected organs, followed by neurological system （ 12% ） and cardiovascular system （ 11% ）. cANCA was positive in 77% of patients, while ANCA was negative in 8%. Images mostly showed multiple nodules/mass with/without cavity in lung （59%） and sinusitis （57%）. The pathologic features were necrotic granulomatosis and/or microvasculitis,which was 78% in nasal mucosa/mass biopsy and 75% in lung.Focal segmental necrotic glomerulonephritis （ 59% ） was an important feature for confirming the diagnosis. Of the WG groups, 49% of patients were in generalized subgroup followed by localized （22%）, early systemic （ 15% ）, severe renal （9%） and refractory group （5%）. The patients were treated with corticosteroid and cyclophosphamide. The remission rate in the induction phase was 78%, while the mortality rate was 4%. The follow-up duration ranged from 1 to 145 months. Complications included infection （22%）, chronic renal failure（ 12% ）, deep venous thrombosis（ 11% ）. Five patients died（8% ）, in which 2 patients died of infection.Conclusion The clinical manifestations of Wegener＇s granulomatosis are complicate. ANCA testing, images of sinus and lung and histological biopsy have played important roles in early diagnosis, which is significant to initiate appropriate and prompt treatment in order to reduce complications and improve prognosis.

关 键 词：韦格纳肉芽肿病 血管炎 中枢神经系统 治疗 亚组分型 

分 类 号：R686[医药卫生—骨科学]