目的探讨ANCA相关血管炎脏器受累与肾功能进展之间的关系。方法对31例ANCA相关性小血管炎患者临床资料进行分析,观察患者肾功能进展情况。结果呼吸系统症状首发患者发病确诊间隔时间明显长于肾损害症状首发患者;初诊肾功能正常合并肺损伤患者治疗1 a后仍有50%出现不同程度肾功能损害。结论合并肺损伤的血管炎患者更易发生肾功能恶化,对于以肺部损伤为临床表现的患者应早期行ANCA检查。 Objective To investigate the relationship between ANCA-associated vasculitis and organ involvement and the progression of renal function. Methods The clinical data of 31 patients with ANCA-associated vasculitis were analyzed and the progress of renal function was observed. Results The onset time of patients with respiratory symptoms was significantly longer than that of the patients with initial symptoms of kidney damage. There were still 50% renal dysfunction in newly diagnosed patients with lung function after 1 year of treatment. Conclusions Patients with vasculitis with lung injury are more likely to have worsening renal function. ANCA should be performed early in patients with lung injury as a clinical manifestation.