Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis.
Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis.
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发表时间：
1996
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通讯作者：
S. Hogan;P. Nachman;Wilkman As;J. Jennette;R. Falk
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作者：
S. Hogan;P. Nachman;Wilkman As;J. Jennette;R. Falk
本研究的目的是确定临床、实验室和病理学特征在抗神经细胞胞质自身抗体（ANCA）相关的显微镜下多血管炎和肾小球肾炎（不包括韦格纳肉芽肿病）患者中对患者和肾脏存活率的预后价值。107例ANCA阳性的坏死性和新月体性肾小球肾炎患者，包括69例有显微镜下多血管炎证据的患者，在本研究中进行了评价。计算以下潜在预后指标的相对死亡风险：（1）ANCA模式;（2）发作时肺出血;（3）存在肾外表现与肾局限性疾病;（4）与单独使用皮质类固醇相比，皮质类固醇和环磷酰胺治疗（静脉或口服）。使用考克斯比例风险模型评估以下变量对肾存活率的预测值：（1）年龄;（2）种族;（3）疾病发作时的肺部症状;（4）肾脏病理学;（5）ANCA模式;和（6）肾活检时获得的血清肌酐峰值。患者前瞻性随访2.5年（范围，5天至12年2个月）。有12例疾病相关死亡，46例患者达到ESRD。与核周型ANCA患者相比，肺出血患者的患者死亡相对风险（和95%置信区间）高8.65（3.36，22.2）倍，胞浆型ANCA患者高3.78（1.22，11.70）倍。肺出血的相对危险度与ANCA类型无差异。环磷酰胺治疗的患者死亡风险比单纯皮质类固醇治疗的患者低5.56倍。肾存活率的预测因子是入组时的血清肌酐值（P = 0.0002）、种族（非裔美国人的预后比白人差，P = 0.0008）、肾活检中存在动脉硬化（P = 0.0076），控制年龄、ANCA模式、显微镜下多血管炎与单纯肾小球肾炎和肺部受累。当控制入组时血清肌酐值、人种和动脉硬化时，病理学指标如肾小球坏死、肾小球新月体、肾小球硬化和间质硬化不能预测肾存活率。然而，在肌酐峰值≤ 3.0 mg/dL的患者亚组（N = 29）中，间质硬化增加是肾脏预后不良的预测因子（P = 0.04）。
The purpose of this study was to determine the prognostic value of clinical, laboratory, and pathologic features at the time of presentation on patient and renal survival in patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated microscopic polyangiitis and glomerulonephritis (excluding Wegener's granulomatosis). One hundred seven ANCA-positive patients with necrotizing and crescentic glomerulonephritis, including 69 with evidence for microscopic polyangiitis, were evaluated for this study. The relative risk of death was calculated for the following potential prognostic indicators: (1) ANCA pattern; (2) pulmonary hemorrhage at onset; (3) presence of extrarenal manifestations versus renal limited disease; and (4) treatment with corticosteroids and cyclophosphamide (intravenous or oral), compared with corticosteroids alone. Cox's proportional hazard model was used to assess the predictive value of the following variables on renal survival: (1) age; (2) race; (3) pulmonary symptoms at onset of disease; (4) renal pathology; (5) ANCA pattern; and (6) peak serum creatinine values obtained near the time of renal biopsy. Patients were followed prospectively for 2.5 yr (range, 5 days to 12 yr 2 months). There were 12 disease-related deaths and 46 patients who reached ESRD. The relative risk (and 95% confidence interval) of patient death was 8.65 (3.36, 22.2) times greater in patients who presented with pulmonary hemorrhage, and 3.78 (1.22, 11.70) times greater in patients with cytoplasmic ANCA compared to those with perinuclear ANCA. The relative risk of pulmonary hemorrhage was no different by ANCA pattern. The risk of death was 5.56 times lower in the cyclophosphamide-treated patients versus those treated with corticosteroids alone. The predictors of renal survival were entry serum creatinine value (P = 0.0002), race (African Americans having a worse outcome compared with Caucasians, P = 0.0008), and the presence of arterial sclerosis on kidney biopsy (P = 0.0076) when controlling for age, ANCA pattern, microscopic polyangiitis versus glomerulonephritis alone, and pulmonary involvement. Pathology indices such as glomerular necrosis, glomerular crescents, glomerular sclerosis, and interstitial sclerosis were not predictive of renal survival when controlling for entry serum creatinine value, race, and arterial sclerosis. However, in the subgroup of patients with a peak creatinine value of < or = 3.0 mg/dL (N = 29), increased interstitial sclerosis was a predictor of a poor renal outcome (P = 0.04).