Huang Jinxian, Wu Ling, Huang Xiaoyan, Xie Yan, Yu Jinquan, Yang Jin, Fang Huiqiong, Zhang Lijun
Rheumatology Department, The University of Hong Kong Shenzhen Hospital, Shenzhen, China.
Case Rep Nephrol Dial. 2016 Jan 8;6(1):1-7. doi: 10.1159/000443163. eCollection 2016 Jan-Apr.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and anti-glomerular basement membrane (GBM) disease are two separate diseases, while sometimes they can coexist together. The exact mechanisms are not clear, but due to the rapid progression and poor prognosis, prompt and aggressive treatment is usually required. We treated with steroids combined with cyclophosphamide and rituximab an 84-year-old man with ANCA-associated vasculitis and anti-GBM disease who had prior pulmonary fibrosis and a coexisting anterosuperior mediastinal mass. Conventional therapy including steroids, plasmapheresis and cyclophosphamide failed to attenuate the anti-GBM disease, yet he responded to an alternative treatment of rituximab. This case suggests the efficacy of steroids and immunosuppressant for the treatment of a dual-positive case with an anterosuperior mediastinal mass.
抗中性粒细胞胞浆抗体（ANCA）相关血管炎和抗肾小球基底膜（GBM）病是两种不同的疾病，但有时可同时存在。确切机制尚不清楚，但由于病情进展迅速且预后较差，通常需要及时且积极的治疗。我们用类固醇联合环磷酰胺和利妥昔单抗治疗了一名84岁患有ANCA相关血管炎和抗GBM病的男性患者，该患者既往有肺纤维化且同时存在前上纵隔肿块。包括类固醇、血浆置换和环磷酰胺在内的传统治疗未能减轻抗GBM病，但他对利妥昔单抗的替代治疗有反应。该病例提示了类固醇和免疫抑制剂对治疗伴有前上纵隔肿块的双阳性病例的疗效。
