成人薄基底膜肾小球病的临床病理特点及预后分析

Clinico-pathological characteristics and outcomes in adult patients with thin basement membrane nephropathy

目的探讨成人薄基底膜肾小球病(thin basement membrane nephropathy,TBMN)的临床病理特征及预后。方法选取2008年1月1日至2017年12月31日在东部战区总医院肾脏科经肾活检确诊为TBMN的成年患者,回顾性分析临床、病理特点和预后,以及蛋白尿和肾脏慢性病理改变的影响因素。结果共纳入135例TBMN患者,女性116例(85.9%),男性19例(14.1%),年龄(40.56±10.30)岁,30例(22.2%)合并高血压,32例(23.7%)合并超重/肥胖。135例患者均有镜下血尿,血清肌酐均正常,41例(30.4%)存在蛋白尿[(0.65±0.19)g/24 h]。102例(75.6%)肾脏病理可见肾小球硬化,其中51例(37.8%)球性硬化比例>10%。79例(58.5%)存在轻度肾小管间质慢性病变,53例(39.3%)存在血管透明变性。合并超重/肥胖和(或)高血压患者的蛋白尿、肾小管间质慢性病变及肾血管病变比例均高于无合并症组(均P<0.05)。多因素Logistic回归分析结果显示,超重/肥胖(OR=7.550,95%CI 2.091~27.257,P=0.002)和高血压(OR=4.424,95%CI 1.091~17.935,P=0.037)是蛋白尿的独立影响因素,蛋白尿是肾小管间质慢性病变的独立影响因素(OR=3.151,95%CI 1.046~9.491,P=0.041)。4例患者失随访,余患者中位随访时间为64.0(24.0,96.5)个月。随访末10例尿蛋白增加,3例(2.2%)出现估算肾小球滤过率下降,此13例患者均合并超重/肥胖,其中4例伴高血压,余118例患者尿检较基线无明显变化,肾功能正常。结论成人TBMN患者蛋白尿、肾脏慢性病变发生率高,合并超重/肥胖、高血压可能造成预后不良,未合并代谢异常的TBMN患者远期预后良好,但尚需长期随访观察。

Objective To investigate the clinico-pathological characteristics and outcomes of adult patients with thin basement membrane nephropathy(TBMN).Methods Patients with biopsy-proven TBMN in National Clinical Research Center of Kidney Diseases,Jinling Hospital during Jan 1,2008 to Dec 31,2017 were collected.The clinico-pathological characteristics,prognosis,the influencing factors of proteinuria and renal chronic lesions were retrospectively analyzed.Results Among 135 adult patients included,116 cases(85.9%)were female,and 19 cases were males.The age was(40.56±10.30)years old.There were 30 cases(22.2%)with hypertension and 32 cases(23.7%)with overweight or obesity.Proteinuria was found in 41 patients(30.4%)with(0.65±0.19)g/24 h of urine protein,and microscopic hematuria was found in all 135 patients.Serum creatinine was normal in all patients.Glomerulosclerosis was observed in 102 cases(75.6%),in which 51 cases(37.8%)had glomerulosclerosis>10%.There were 79 cases(58.5%)with mild chronic tubulointerstitial lesions,and 53 patients(39.3%)with vascular hyalinosis.The proportions of proteinuria,chronic tubular interstitial lesion and renal vascular lesion in patients with overweight/obesity and/or hypertension were higher than those without complications(all P<0.05).Multivariate logistic regression results showed that overweight/obesity(OR=7.550,95%CI 2.091-27.257,P=0.002)and hypertension(OR=4.424,95%CI 1.091-17.935,P=0.037)were independent influencing factors for proteinuria,while proteinuria was the independent influencing factor for chronic tubular interstitial lesion(OR=3.151,95%CI 1.046-9.491,P=0.041).Four patients were lost to follow-up,and the median follow-up time of the remaining patients was 64.0(24.0,96.5)months.At the end of the follow-up,urine protein increased in 10 patients(7.4%)and estimated glomerular filtration rate decreased in 3 patients(2.2%).The above 13 cases were all complicated with overweight/obesity and 4 cases with hypertension.The urine test and renal function in the remaining 118 patients didn't change significantly from baseline.Conclusions The incidences of proteinuria and renal chronic lesion are high in adult TBMN patients.Overweight/obesity and hypertension may cause a poor prognosis,and TBMN patients without metabolic abnormalities probably have good prognosis,but need long-term follow-up.