坏死性肉芽肿性血管炎20例临床分析

Clinical analysis of 20 patients with necrotizing granulomatous vasculitis

目的 分析坏死性肉芽肿性血管炎（NGV ）的临床资料.方法对南京鼓楼医院收治的20例NGV患者的临床资料进行分析.结果 男11例,女9例,发病年龄18~76岁,平均年龄44.7岁.可累及多个系统或器官,以肺脏受累最常见,占100%,其次为鼻部和肾脏,各为85%.血抗中性粒细胞胞质抗体（ANCA）阳性率95%.影像学以肺部多发结节/肿块影伴或不伴空洞病灶（65%）为主.最常见的病理表现为坏死性肉芽肿和小血管炎.结论 NGV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.

Objective To analyze the clinical data of necrotizing granulomatous vasculitis （NGV）. Methods The clinical data of 20 patients with NGV were analyzed. Results There were 11 males and 9 females. The onset age ranged from 18 to 76 years old, and their mean age was 44.7 years old. NGV often had multiple system involvement. Lung was the major affected organ （100%）, followed by upper respiratory tract （85%） and kidney （85%）. The positive rate of anti-neutrophil cytoplasmic antibody （ANCA） was 95%. Images mostly showed multiple nodules and mass with/without cavity in lung （65%）. The pathologic features included necrotizing granulomatosis and microvasculitis. Conclusion The clinical manifestations of NGV are complicate. ANCA testing, images of sinus and lung and histological biopsy have played important roles in the early diagnosis and treatment.