PDF(Pubmed)
Abstract：
Background: The spectrum and outcomes of crescentic glomerulonephritis (Cr.GN) in South Asia is vastly different from that reported worldwide and there is a paucity of information. The aim of the study was to study the demography, clinical presentation, histology and predictors of longitudinal outcomes of Cr.GN in this population. Methods: An observational cohort study of renal biopsies was performed in the largest tertiary center in South India over a period of 10 years (January 2006 to December 2015) with ≥50% crescents on renal histology indicating Cr.GN. Results: A total of 8645 kidney biopsies were done; 200 (2.31%) were Cr.GN. Patients were categorized into three etiological groups: anti-glomerular basement membrane (type I), immune complex (type II), and pauci-immune (type III). Type II was the most common (96, 46.5%), followed by type III (73, 38%) and type I (31, 15.5%). Female preponderance was seen across all types. About half of all patients presented with recent onset hypertension. Type II had the highest median proteinuria (4.2 (2.1-6) g/day, p=0.06) and the median estimated glomerular filtration rate was lowest in type I (5 (4-8) ml/min/1.73m 2, p<0.001). Among type III, anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis was seen only in ~50% of patients. Nearly one third of patients with type I were also positive for ANCA making them \'double positive\'. Acute glomerular insults like tuft necrosis and chronic changes as evidenced by moderate to severe interstitial fibrosis, was a predominant feature of type I. Conclusions: ANCA-negative pauci-immune vasculitis, as well as double positive Cr.GN, are reported for the first time in South-Asia. Renal survival was significantly worse in type I/III compared to type II. Types I/III, moderate to severe interstitial fibrosis and tubular atrophy, presence of oliguria/anuria and increasing percentage of crescents in renal biopsy were significant predictors of end stage kidney disease in our cohort.
摘要：
背景：新月体肾小球肾炎的频谱和结局（Cr。南亚的GN）与全球报告的情况大不相同，信息匮乏。这项研究的目的是研究人口统计学，临床表现,Cr纵向结局的组织学和预测因子。GN在这个人口中。方法：在印度南部最大的三级中心进行了为期10年（2006年1月至2015年12月）的肾活检观察性队列研究，肾脏组织学上新月≥50％提示Cr。GN.结果：共进行了8645例肾脏活检；200例（2.31％）为Cr。GN.患者分为三个病因组：抗肾小球基底膜（I型），免疫复合物（II型），和免疫（III型）。II型是最常见的（96，46.5％），其次是III型（73，38％）和I型（31，15.5％）。所有类型都有女性优势。所有患者中约有一半患有近期发作的高血压。II型蛋白尿中位数最高（4.2（2.1-6）g/天，p=0.06），估计的肾小球滤过率中位数在I型中最低（5（4-8）ml/min/1.73m2，p<0.001）。在III型中，抗中性粒细胞胞浆抗体(ANCA)相关性血管炎仅见于约50%的患者.近三分之一的I型患者的ANCA也呈阳性，使他们“双阳性”。急性肾小球损伤，如簇状坏死和慢性改变，如中度至重度间质纤维化证明，是I型的主要特征。结论：ANCA阴性的免疫性血管炎，以及双正Cr。GN,这是南亚首次报道。与II型相比，I/III型的肾脏存活率明显更差。I/III型,中度至重度间质纤维化和肾小管萎缩，在我们的队列中,少尿/无尿的存在和肾活检中新月体百分比的增加是终末期肾病的重要预测因子.
