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目的：探讨局限型韦格纳肉芽肿病（Wegener Granulomatosis,WG）肺部影像学表现及病理学特征，以提高对本病的认识。方法报告1例肺局限型韦格纳肉芽肿的临床及影像学资料，行HE及特殊染色，分析其临床、影像、病理学特征，并复习相关文献。结果患者中年女性，临床症状因活动后出现胸背部隐痛、气促、乏力。胸部CT示双肺多发团块及结节状密度增高影，伴空洞形成。其它脏器检查均未发现异常。镜下肺组织内见大量肉芽肿性结节，结节中央可见地图状坏死或中性粒细胞呈微脓肿样聚集和血管炎。特殊染色：抗酸染色（-）、六胺银染色（-）、血管壁弹力纤维染色（少量-）。结论局限型WG病变仅限于肺，基本病理改变为坏死性肉芽肿、微脓肿和血管炎。对糖皮质激素和环磷酰胺联合用药治疗效果明显。“,”Objective To investigate the clinical,radiographic and pathologic features of Wegener granulomatosis (WG)for the sake of bet er understanding of this diseases. Methods By reporting a case of restrictive Wegener granulomatosis of lung and analyzing its clinicopathologic features in HE sections and special stains.We also reviewed associated documents. Results a mid-age woman who shows moderate pain in back and chest,with breathless,fatigue after exertion.CT demonstrated that multiple masses and nodular high density area with cavitation in both lungs. There are no abnormal changes in other organs. Histological y,irregular necrosis or aggregated neutrophils which like microabscess can be seen in central area of granuloma, vasolititis is another evident of this disease. Special stains: acid-fast stain and silver stain were both negative, elastic fiber stain is positive in most area. Conclusion restrictive WG is confined in lung,its basic alteration are necrotizing granulomas,microabscess and vasolitis, which shows apparent response to cortical steroids combined with cyclophosphamide.