Mungan Sevdegul, Turkmen Ercan, Aydin Makbule Cisel, Saglam Arzu Emine, Baydar Dilek Ertoy
a Department of Pathology , Karadeniz Technical University, School of Medicine , Trabzon , Turkey .
Ren Fail. 2015 Jun;37(5):858-65. doi: 10.3109/0886022X.2015.1033635. Epub 2015 Apr 10.
The glomerular tip lesion (GTL) is a distinctive histopathologic lesion which is regarded as a variant of focal and segmental glomerulosclerosis (FSGS). The prognostic significance of GTL among other FSGS variants has been disputed. In order to define the clinical features and outcome of GTL, we retrospectively reviewed the presenting clinical features, laboratory and biopsy findings and surveillance in our cohort of GTL, which consisted of 20 adults with native kidneys (mean age 46 years) with follow-up data ranging from 3 to 137 months. At presentation, mean urine protein, serum albumin and cholesterol levels were 5.17 g/d, 2.6 g/dL and 312.9 mg/dL, respectively, and none had renal insufficiency. Microscopic hematuria was detected in five patients. At biopsy, glomerular segmental lesions consisted of GTL without perihilar or collapsing lesions. GTL was observed in a variable proportion of glomeruli from 2.6% to 100%. Mesangial proliferation was seen in nine cases, at a moderate degree in two and mild in the rest. Three biopsies showed mild, two showed moderate interstitial fibrosis/tubular atrophy. Eleven patients received steroids alone and eight received sequential therapy with steroids and a cytotoxic agent. At a mean follow-up of 40.6 months, 17 patients (85%) achieved complete remission of nephrotic syndrome, 15% had partial remission. Four of 17 suffered from recurrences. No patient progressed to end-stage renal disease. Serum albumin at diagnosis was the only predictor of a recurrence (p = 0.037). Microscopic hematuria correlated with incomplete remission (p = 0.045). Our study demonstrates a clearly favorable prognosis in patients with FSGS-GTL variant.
肾小球顶端病变（GTL）是一种独特的组织病理学病变，被视为局灶节段性肾小球硬化（FSGS）的一种变体。GTL在其他FSGS变体中的预后意义一直存在争议。为了明确GTL的临床特征和预后，我们回顾性分析了我们队列中GTL患者的临床表现、实验室检查和活检结果以及随访情况，该队列包括20例成年原发性肾脏病患者（平均年龄46岁），随访时间为3至137个月。初诊时，平均尿蛋白、血清白蛋白和胆固醇水平分别为5.17g/d、2.6g/dL和312.9mg/dL，且均无肾功能不全。5例患者检测到镜下血尿。活检时，肾小球节段性病变由无肾门周围或塌陷性病变的GTL组成。GTL在不同比例的肾小球中被观察到，范围从2.6%到100%。9例可见系膜增生，其中2例为中度，其余为轻度。3例活检显示轻度，2例显示中度间质纤维化/肾小管萎缩。11例患者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40.6个月时，17例患者（85%）实现了肾病综合征的完全缓解，15%部分缓解。17例中有4例复发。无患者进展至终末期肾病。诊断时的血清白蛋白是复发的唯一预测因素（p = 0.037）。镜下血尿与不完全缓解相关（p = 0.045）。我们的研究表明，FSGS-GTL变体患者的预后明显良好。
