Objective To investigate the clinical features of patient with Wegener granulomatosis ( WG) complicated with pulmonary infection. Methods Twenty three patients with WG complicated with pulmonary infection admitted to Peking Union Medical College Hospital in 12 years were retrospectively analyzed. Results Among 23 patients, 21.7% pulmonary infection occurred at the first diagnosis, and 52.2% occurred within 6 months, while 26. 1 % occurred more than 6 months. 73. 9% were generalized, and 26.1% were localized, accompanied with erythrocyte sedimentation rate ( ESR, 63.6%), C-reactionprotein ( CRP, 65%) increased, and immunoglobulin (30%) decreased. Antineutrophil cytoplasmic antibodies (ANCA) was positive in 82. 6%, and C-ANCA was positive in 69. 6% . The clinical manifestations of pulmonary infection were productive cough ( 86.9%), fever and fatigue(52. 2% ), hemoptysis (52. 2% ) , chest pain and pactoralgia (34. 8% ). The causative pathogen mainly were bacteria (56.5%), tubercle bacillus (43.5%), and fungas (39.1%). Diabetes mellitus or impaired glucose tolerance (34.8%) and hypoproteinemia (65.2%) were the common complication. Most patients (95.7%) were treated with corticosteroid and cyclophosphamide, 21.7% receive methyllprednisolone pulse therapy. Three patients died. Conclusions Patients with WG tend to complicate with pulmonary infection in the first six months, most of whom were generalized. The clinical manifestations were productive cough. The causative pathogens mainly were bacteria, tubercle bacillus, and fungas. Most patients were treated with corticosteroid and cyclophosphamide.%目的:了解韦格纳肉芽肿(WG)合并肺部感染的临床特点.方法:回顾性分析北京协和医院1998年1月至2010年12月收治的23例WG合并肺部感染患者的临床特点及治疗转归.结果:23例患者中首次就诊即合并肺部感染5例,占21.7%;确诊WG 6个月内发生肺部感染12例,占52.2%;确诊WG 6个月以上发生肺部感染6例,占26.1%.全身型73.9%,局限型26.1%;红细胞沉降率(63.6%)、C反应蛋白(65%)升高,免疫球蛋白(30%)减低.抗中性粒细胞胞浆抗体(ANCA)阳性率82.6%,C-ANCA阳性率69.6%.肺部感染表现为咳嗽、咳痰(86.9%),发热、乏力(52.2%),咯血(52.2%),胸闷、胸痛(34.8%).主要为细菌感染(56.5%),结核分支杆菌感染(43.5%),真菌感染(39.1%).15例(65.2%)合并低蛋白血症,8例(34.8%)合并糖尿病或糖耐量减低.22例接受糖皮质激素联合环磷酰胺治疗,其中5例接受甲基泼尼松龙冲击治疗,3例(13.4%)死亡.结论wG合并肺部感染6个月内发生率高,多为全身型,其临床上表现为咳嗽、咳痰,病原学检查细菌、结核分支杆菌、真菌感染多见,绝大部分患者使用过激素、免疫抑制剂治疗.
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