Stokes M Barry, Markowitz Glen S, Lin Julie, Valeri Anthony M, D'Agati Vivette D
Department of Pathology, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA.
Kidney Int. 2004 May;65(5):1690-702. doi: 10.1111/j.1523-1755.2004.00563.x.
The glomerular tip lesion (GTL) is a distinctive but controversial histopathologic lesion occurring in patients with idiopathic nephrotic syndrome. The relationship of GTL to minimal change disease (MCD) and idiopathic focal segmental glomerulosclerosis (FSGS) has been disputed.
In order to define the clinical features and natural history of GTL, we retrospectively reviewed the presenting clinical features, biopsy findings and outcome of 47 cases. Presenting clinical features of GTL were compared to those of controls with MCD (N= 61) or idiopathic FSGS (N= 50).
The cohort of GTL consisted of 45 adults and two children (mean age 47.5 years; range 12 to 79 years), including 76.6% Caucasians and 53% males. At presentation, 93.6% of patients had edema, 89.1% had nephrotic syndrome (mean urine protein 8.31 g, mean serum albumin 2.27 g/dL, and mean cholesterol 340.6 mg/dL), and 34.8% had renal insufficiency. Mean time from onset of renal disease to biopsy was 2.4 months. At biopsy, glomerular segmental lesions included GTL alone in 26%, GTL and peripheral lesions in 6%, GTL and indeterminate lesions in 36%, and GTL with peripheral and indeterminate lesions in 32%. No initial biopsy contained perihilar sclerosis and most (81%) segmental lesions were cellular. Follow-up data were available in 29 patients, of whom 21 received steroids alone and eight received sequential therapy with steroids and a cytotoxic agent. At a mean follow-up of 21.5 months, 58.6% of patients achieved complete remission of nephrotic syndrome, 13.8% had partial remission, and 27.6% had persistent nephrotic proteinuria. Only one patient progressed to end-stage renal disease (ESRD). Predictors of nonremission included severity of proteinuria at presentation and % peripheral lesions. When compared to controls with MCD and idiopathic FSGS, GTL more closely resembled MCD with respect to high incidence of nephrotic syndrome (P < 0.001), severity of proteinuria (P < 0.05), short duration from onset to biopsy (P < 0.001), and absence of chronic tubulointerstitial disease (P < 0.0054).
Within the MCD/FSGS spectrum, GTL is a distinctive and prognostically favorable clinical-pathologic entity whose presenting features and outcome more closely approximate those of MCD.
肾小球顶端病变（GTL）是特发性肾病综合征患者中一种独特但存在争议的组织病理学病变。GTL与微小病变性肾病（MCD）和特发性局灶节段性肾小球硬化（FSGS）的关系一直存在争议。
为了明确GTL的临床特征和自然病程，我们回顾性分析了47例患者的临床表现、活检结果及预后。将GTL的临床表现与MCD对照组（N = 61）或特发性FSGS对照组（N = 50）进行比较。
GTL队列包括45例成人和2例儿童（平均年龄47.5岁；范围12至79岁），其中76.6%为白种人，53%为男性。就诊时，93.6%的患者有水肿，89.1%的患者有肾病综合征（平均尿蛋白8.31g，平均血清白蛋白2.27g/dL，平均胆固醇340.6mg/dL），34.8%的患者有肾功能不全。从肾病发病到活检的平均时间为2.4个月。活检时，肾小球节段性病变包括单纯GTL占26%，GTL和外周病变占6%，GTL和不确定病变占36%，GTL伴外周和不确定病变占32%。初次活检均无肾门周围硬化，大多数（81%）节段性病变为细胞性。29例患者有随访数据，其中21例仅接受了类固醇治疗，8例接受了类固醇和细胞毒性药物的序贯治疗。平均随访21.5个月时，58.6%的患者肾病综合征完全缓解，13.8%部分缓解，27.6%持续存在肾病性蛋白尿。只有1例患者进展为终末期肾病（ESRD）。未缓解的预测因素包括就诊时蛋白尿的严重程度和外周病变的比例。与MCD和特发性FSGS对照组相比，GTL在肾病综合征高发病率（P < 0.001）、蛋白尿严重程度（P < 0.05）、发病到活检的短病程（P < 0.001）以及无慢性肾小管间质疾病（P < 0.0054）方面更类似于MCD。
在MCD/FSGS范围内，GTL是一种独特且预后良好的临床病理实体，其临床表现和预后更接近MCD。
