韦格纳肉芽肿病34例临床分析  

Clinical analysis of 34 patients of Wegener＇ s granulomatosis

作　　者：段新旺[1,5] 孟庆良[2,5] 王慧[3,5] 吴爱瑜[4,5] 王迁[5] 李梦涛[5] 曾小峰[5] 

机构地区：[1]南昌大学第一附属医院风湿免疫科,南昌330006 [2]河南省中医院风湿免疫科 [3]内蒙古包头医学院第一附属医院风湿免疫科 [4]福建龙岩市第一医院风湿科 [5]中国医学科学院北京协和医学院北京协和医院风湿免疫科

出　　处：《中华急诊医学杂志》2012年第10期1159-1163,共5页Chinese Journal of Emergency Medicine

摘　　要：目的分析韦格纳肉芽肿病（Wegener’s granulomatosis,WG）患者的临床诊疗资料，提高对本病的认识。方法回顾性研究分析2002年1月至2012年3月在北京协和医院确诊的34例WG患者的临床表现、实验室检查、影像特点、病理特点、治疗及疗效。结果34例患者中，男18例，女16例，发病年龄（45±15）岁（18-81岁）；确诊时间是（140±72）个月，3个月以内确诊率29.4%;首发临床表现多为肺（41.1%）、鼻（38.2%）、眼（11.7%）、头痛（11.7%）、耳（8_8%），非特异症状如发热（26.4%）等亦不少见；全部临床表现多为肺部（76.4%）、鼻（73.5%）、肾脏（67.6%）、眼（58.8%）、耳（47.0%）;神经系统及口腔症状亦不少见，分别是41.1%、20.5%。实验室检查C-ANCA及PR3阳性率61.7%’P-ANCA及MP0阳性率20.5%,ANCA阴性率11.7%，P-ANCA阳性及MPO阴性1例（2.9%）,C-ANCA阳性并PR3及MP0均阳性1例（2.9%）。肺部影像表现复杂多样，多为结节或肿块（34%）、索条影（20.5%）、空洞（17.6%）、斑片影（17.6%）;典型病理三联征表现1例（4.3%）,出现典型病理中二联表现10例（43.4%）;在入院前误诊为肺结核、肺癌、肺脓肿，误诊率为20.5%;肺部感染发生率高达50%;人院后给予激素和环磷酰胺正规诱导缓解及维持治疗，难治型给予血浆置换、静脉输注丙种球蛋白（IVIG）和抗CD20单克隆抗体利妥昔单抗，治疗疗效好。结论WG的临床表现复杂多样且变化迅速，容易误诊误治；早期积极运用糖皮质激素和环磷酰胺疗效好。Objective To analyze the clinical data of diagnosis and treatment of Wegener’s granulomatosis （ WG） in order to understand the nature of this disease. Methods The clinical data including clinical manifestations, laboratory findings imaging features, pathological changes and efficacy of treatment of 34 patients with WG admitted from January 2002 to March 2012 were analyzed. Results Of the 34 patients, male to female ratio was 18 to 16,and the average age subjected to WG onset was （45 ± 15 ） years old ranged from 18 to 81 years old. The average duration before the diagnosis confirmed was （ 140 ± 72） mouths, while 29.4% was diagnosed within 3 months. The presenting symptoms included initial involvements in lung （41. 1% ） , nose （ 38. 2% ） , eyes （11. 7% ）,ear （ 8. 8% ） and constitutional symptoms such as fever （26. 4% ）.Throughout the whole disease course, the incidence of systemic （47. 0% ） , nervous system （41. 1% ） , oral ulcer （20. 5% ）. Of laboratory findings, C - ANCA/PR3 -ANCA was positive in 61.1% patients, P - ANCA/MPO - ANCA positive in 20. 5% patients, ANCA negative in 11. 7% patients, P -- ANCA positive with MPO - ANCA negative in one patient （2. 9 % ） and C - ANCA positive with PR3 and MPO positive in one patient （2. 9% ）. Of imaging findings, nodules or masses were most commonly observed （ 34% ）, followed by fibrotic lesions （ 20. 5% ） , cavitations （17. 6% ）、opacities （17. 6% ） . Typically pathological triad （ vasculitis, necross and granuloma formation） was found in one patient （4. 3% ） , while two pathological changes of the triad occurred in 10 patients （43.4%）. Pulmonary infection was highly prevalent （50%）,and of them, 20.5% patients were misdiagnosed as tuberculosis, malignancy or abscess. After admission, high dose of corticosteroids and cyclophosphamide were administered as bolus and maintenance therapy. Plasma exchange, intravenous immunoglobulin,and rituximab were added for refractory cases and optimal

关 键 词：韦格纳肉芽肿病 血管炎 肺部影像 抗中性粒细胞胞浆抗体 病理特点 感染 确诊时间 治疗 

分 类 号：R593.9[医药卫生—内科学]