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Anti-Glomerular Basement Membrane Disease: Outcomes of Different Therapeutic Regimens in a Large Single-Center Chinese Cohort Study

机译:抗肾小球基底膜疾病:大型单中心中国队列研究中不同治疗方案的结果。

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Anti-glomerular basement membrane (GBM) disease usually presents with rapidly progressive glomerulonephritis accompanied by pulmonary hemorrhage. The low incidence and fulminant course of disease preclude a large randomized controlled study to define the benefits of any given therapy. We conducted a retrospective survey of 221 consecutive patients seen from 1998 to 2008 in our hospital, and report here the patient and renal survival and the risk factors affecting the outcomes. Considering the similar clinical features of the patients, we could compare the effects of 3 different treatment regimens: 1) combination therapy of plasmapheresis and immunosuppression, 2) steroids and cytotoxic agents, and 3) steroids alone. The patient and renal survival rates were 72.7% and 25.0%, respectively, at 1 year after disease presentation. The serum level of anti-GBM antibodies (increased by 20 U/mL; hazard ratio [HR], 1.16; p = 0.009) and the presentation of positive antineutrophil cytoplasmic antibodies (ANCA) (HR, 2.18; p = 0.028) were independent predictors for patient death. The serum creatinine at presentation (doubling from 1.5 mg/dL; HR, 2.07; p The combination therapy of plasmapheresis plus corticosteroids and cyclophosphamide had an overall beneficial effect on both patient survival (HR for patient mortality, 0.31; p = 0.001) and renal survival (HR for renal failure, 0.60; p = 0.032), particularly patient survival for those with Goodpasture syndrome (HR for patient mortality, 0.29; p = 0.004) and renal survival for those with anti-GBM nephritis with initial serum creatinine over 6.8 mg/dL (HR for renal failure, 0.52; p = 0.014). The treatment with corticosteroids plus cyclophosphamide was found not to improve the renal outcome of disease (p = 0.73). In conclusion, the combination therapy was preferred for patients with anti-GBM disease, especially those with pulmonary hemorrhage or severe renal damage. Early diagnosis was crucial to improving outcomes. Abbreviations: ANCA = antineutrophil cytoplasmic antibodies, CI = confidence interval, ELISA = enzyme-linked immunosorbent assay, ESRD = end-stage renal disease, GBM = glomerular basement membrane, HR = hazard ratio.
机译:抗肾小球基底膜(GBM)疾病通常表现为快速进行性肾小球肾炎,并伴有肺出血。疾病的低发病率和暴发性病程妨碍了一项大型随机对照研究来确定任何给定治疗的益处。我们对1998年至2008年在我院就诊的221例连续患者进行了回顾性调查,并在此报告了患者和肾脏的存活率以及影响预后的危险因素。考虑到患者的相似临床特征,我们可以比较3种不同治疗方案的效果:1)血浆置换和免疫抑制的联合治疗; 2)类固醇和细胞毒剂,以及3)类固醇。发病后1年,患者和肾脏的存活率分别为72.7%和25.0%。抗GBM抗体的血清水平(增加20 U / mL;危险比[HR],1.16; p = 0.009)和呈阳性的抗中性粒细胞胞浆抗体(ANCA)的呈递(HR,2.18; p = 0.028)是独立的患者死亡的预测指标。出现时的血清肌酐(从1.5 mg / dL翻倍; HR,2.07; p)血浆置换加皮质类固醇和环磷酰胺的联合治疗对患者生存(HR对患者死亡率为0.31; p = 0.001)和肾脏均具有总体有益作用存活率(肾衰竭的HR,0.60; p = 0.032),尤其是Goodpasture综合征患者的存活率(HR,患者死亡率,0.29; p = 0.004),以及抗-GBM肾炎且血清肌酐高于6.8的患者的肾存活率mg / dL(肾衰竭的心率,0.52; p = 0.014),发现使用糖皮质激素加环磷酰胺治疗不能改善肾脏疾病的预后(p = 0.73),总的来说,抗心衰患者首选联合治疗-GBM疾病,尤其是那些患有肺出血或严重肾脏损害的疾病,早期诊断对改善转归至关重要。缩写:ANCA =抗中性粒细胞胞浆抗体,CI =置信区间,ELISA =酶e-联免疫吸附试验,ESRD =晚期肾脏疾病,GBM =肾小球基底膜,HR =危险比。

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