Shah Shivani, Havill John, Rahman M Hafizur, Geetha Duvuru
Division of Nephrology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD, USA.
Division of Nephrology, Department of Medicine, Johns Hopkins Bayview Medical Center, 301 Mason Lord Drive, Suite 2500, Baltimore, MD, 21224, USA.
Clin Rheumatol. 2016 Apr;35(4):953-60. doi: 10.1007/s10067-015-3086-8. Epub 2015 Oct 7.
Anti-neutrophil cytoplasmic antibodies (ANCA) play an important role in the pathogenesis of ANCA-associated vasculitis. The lack of ANCA antibodies may indicate a variation in clinical presentation and outcomes of this disease. We identified 74 adult patients between 1995 and 2009 with the diagnosis of pauci-immune glomerulonephritis. Demographics, histological features, and treatment outcomes were compared between ANCA-positive and ANCA-negative patients. These factors were correlated with renal function at presentation and follow-up. Of the 74 patients, 57 were ANCA-positive, and 17 were ANCA-negative. Demographics and mean Birmingham Vasculitis Activity Score were similar between ANCA-negative and ANCA-positive patients at presentation. Renal function was significantly worse at presentation in the ANCA-negative patients (eGFR 16.59 vs. 31.89 ml/min/1.73 m(2), p = 0.03). Patients in the ANCA-negative group had a significantly higher interstitial fibrosis score compared to the ANCA-positive group (2.1 vs.1.6, p = 0.04). The median time to remission was shorter in the ANCA-negative patients (51 vs. 78 days, p = 0.01). Long-term renal function and 1-year patient and renal survival were similar between ANCA-negative and ANCA-positive patients. Baseline eGFR, percentage of normal glomeruli, glomerular sclerosis, and tubulointerstitial scarring predicted eGFR at 1 year in both groups similarly. This is the first historical review of American patients with pauci-immune glomerulonephritis, comparing patients with ANCA-negative and ANCA-positive serology. Although ANCA-negative patients present with lower eGFR and more interstitial fibrosis, 1-year and long-term outcomes in both groups are similar.
抗中性粒细胞胞浆抗体（ANCA）在ANCA相关性血管炎的发病机制中起重要作用。缺乏ANCA抗体可能表明该疾病的临床表现和预后存在差异。我们确定了1995年至2009年间74例诊断为寡免疫性肾小球肾炎的成年患者。比较了ANCA阳性和ANCA阴性患者的人口统计学、组织学特征和治疗结果。这些因素与就诊时和随访时的肾功能相关。74例患者中，57例ANCA阳性，17例ANCA阴性。就诊时，ANCA阴性和ANCA阳性患者的人口统计学和平均伯明翰血管炎活动评分相似。ANCA阴性患者就诊时的肾功能明显更差（估算肾小球滤过率分别为16.59和31.89ml/min/1.73m²，p = 0.03）。与ANCA阳性组相比，ANCA阴性组患者的间质纤维化评分显著更高（2.1对1.6，p = 0.04）。ANCA阴性患者的缓解中位时间更短（51天对78天，p = 0.01）。ANCA阴性和ANCA阳性患者的长期肾功能以及1年患者生存率和肾脏生存率相似。两组的基线估算肾小球滤过率、正常肾小球百分比、肾小球硬化和肾小管间质瘢痕形成对1年时估算肾小球滤过率的预测相似。这是对美国寡免疫性肾小球肾炎患者的首次历史性回顾，比较了ANCA阴性和ANCA阳性血清学的患者。尽管ANCA阴性患者的估算肾小球滤过率较低且间质纤维化更多，但两组的1年和长期预后相似。
