CLINICAL COURSE OF ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODY-ASSOCIATED GLOMERULONEPHRITIS AND SYSTEMIC VASCULITIS
CLINICAL COURSE OF ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODY-ASSOCIATED GLOMERULONEPHRITIS AND SYSTEMIC VASCULITIS
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DOI：
10.7326/0003-4819-113-9-656
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发表时间：
1990-11-01
影响因子：
39.2
通讯作者：
JENNETTE, JC
中科院分区：
文献类型：
--
作者：
FALK, RJ;HOGAN, S;JENNETTE, JC
目的：确定抗中性粒细胞胞浆抗体（ANCA）相关性肾小球肾炎患者的临床表现谱;确定这些患者的肾脏和患者生存率;比较单独使用皮质类固醇、皮质类固醇+静脉环磷酰胺或皮质类固醇+口服环磷酰胺治疗患者的生存率;并评估疾病表现和治疗反应与ANCA亚型和系列自身抗体滴度的相关性。设计：初始队列研究;平均随访24个月。设置：120所大学和私人执业肾病学家的协作网络（肾小球疾病协作网络）。参会人员：70例ANCA和少免疫性坏死性和新月体性肾小球肾炎患者，其中59例接受皮质类固醇单独治疗（14例患者）、皮质类固醇联合口服环磷酰胺治疗（30例患者）或皮质类固醇联合静脉环磷酰胺治疗（15例患者）。主要结果：70例患者中，18例为肾局限性疾病（特发性新月体肾小球肾炎）; 15例为非肺性肾外疾病，与结节性多动脉炎一致; 37例为肺部疾病，与韦格纳肉芽肿病或肺泡毛细血管炎一致。在产生核周模式的自身抗体的患者之间存在疾病的重叠表现;然而，核周模式在肾局限性疾病患者中更常见。在24个月时，肾脏和患者的生存率为75%，肾限制性疾病患者和全身性疾病患者的生存率无差异。口服环磷酰胺治疗的患者和静脉环磷酰胺治疗的患者的生存率无差异;然而，单独使用皮质类固醇治疗的患者的比较数据不确定。一般来说，自身抗体滴度与治疗反应和疾病活动相关，但也有例外。结论：ANCA患者有各种形式的坏死性血管炎症，从肾局限性疾病到广泛的系统性血管炎，包括结节性多动脉炎和韦格纳肉芽肿病。口服糖皮质激素与口服或静脉注射环磷酰胺治疗ANCA相关性肾小球肾炎似乎同样有效。
Objectives: To determine the spectrum of clinical manifestations in patients with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis; to determine renal and patient survival in these patients; to compare survival among patients treated with corticosteroids alone, corticosteroids plus intravenous cyclophosphamide or corticosteroids plus oral cyclophosphamide; and to assess the correlation of disease manifestations and treatment response with ANCA subtypes and serial autoantibody titers. Design: Inception cohort study; mean follow-up of 24 months. Setting: Collabortative network of 120 university and private practice nephrologists (The Glomerular Disease Collaborative Network). Participants: Seventy patients with ANCA and pauci-immune necrotizing and crescentic glomerulonephritis, of whom 59 were treated with either corticosteroids alone (14 patients), corticosteroids plus oral cyclophosphamide (30 patients), or corticosteroids plus intravenous cyclophosphamide (15 patients). Main results: Of the 70 patients, 18 had renal-limited disease (idiopathic crescentic glomerulonephritis); 15, nonpulmonary extrarenal disease consistent with polyarteritis nodosa; and 37, pulmonary disease consistent with Wegener granulomatosis or alveolar capillaritis. There were overlapping manifestations of disease between patients with autoantibodies producing a perinuclear pattern; however, the perinuclear pattern occurred more frequently in patients with renal-limited disease. Renal and patient survival was 75% at 24 months, and no difference in survival was seen between patients with renal-limited disease and those with systemic disease. No differences in survival were seen between patients treated with oral cyclophosphamide and those treated with intravenous cyclophosphamide; however, the comparative data from patients treated with corticosteroids alone were inconclusive. In general, autoantibody titers correlated with response to treatment and disease activity, but there were exceptions. Conclusions: Patients with ANCA have various forms of necrotizing vascular inflammation, ranging from renal-limited disease to widespread systemic vasculitis, including polyarteritis nodosa and Wegener granulomatosis. Oral corticosteroids with either oral or intravenous cyclophosphamide appear to be equally effective therapy for ANCA-associated glomerulonephritis.