Title: Clinicopathological Features of Idiopathic Membranous Nephropathy in 33 Adolescents. Author: Li C, Li H, Wen YB, Li JN, Lin WF, Cai JF, Duan L, Li Y, Li XM, Li XW. Journal: Zhongguo Yi Xue Ke Xue Yuan Xue Bao; 2017 Aug 20; 39(4):544-551. PubMed ID: 28877834. Abstract: Objective To investigate the clinicopathological features and prognosis of idiopathic membranous nephropathy(IMN)in adolescents. Methods This was a retrospective study on IMN patients hospitalized between June 2012 and December 2014,and a total of 33 IMN patients aged between 13 and 24 years old were enrolled in the study.Meanwhile,33 IMN patients aged more than 24 years old were selected randomly as control group during the same period.Diagnosis was confirmed by renal biopsy,and the secondary causes of membranous nephropathy were ruled out.Data collected from medical record and biopsy were analyzed. Results In the adolescent IMN group,the mean age at renal biopsy was(20±3)years old,and the male/female ratio was 22/11.Twenty-three cases presented as nephrotic syndrome.Systolic and diastolic pressures were(127±13)mmHg and(77±9)mmHg,respectively.The median 24-hour urine protein was 5.14(3.39,9.31)g/d,and the median serum creatinine was 62(52,73)μmol/L.The positive rate of serum anti-phospholipase A2 receptor in adolescent group was 54%.Compared with control group,the adolescent patients had lower incidence of hypertension and higher baseline estimated glomerular filtration rate level [15.2% vs.39.3%,χ2=4.889,P=0.03;125 ml/(min·1.73m2)vs.100 ml/(min·1.73m2),U=137.5,P<0.001].According to IMN staging criteria in electron microscopy,adolescent patients were classified as one case in stage I,21 in stage Ⅱ,and 11 in stage Ⅲ or higher.The positive rates of IgG1,IgG2,IgG3 and IgG4 subclass staining in glomeruli were 46.9%,3.1%,56.3%,and 87.5%,respectively.Compared with control group,the adolescent patients had lower incidence of renal interstitial fibrosis and arteriolar lesions(6.1% vs.66.7%,χ2=26.19,P<0.001;15.2% vs.66.7%,χ2=18.11,P<0.001).Three patients lost to follow-up while others started steroid combined with cyclosporine A(n=20),cyclophosphamide(n=7),or mycophenolate(n=1)or solely(n=2).After a median follow-up of 18(12,24)months,the median proteinuria decreased to 0.20(0.10,0.42)g/d,whereas serum creatinine level remained stable [69(56.8,81.3)μmol/L].Seventeen patients(56.7%)achieved complete remission(CR),and the remaining 13 patients(43.4%)achieved partial remission(PR).The median time of CR and PR were three and six months,respectively.Only one patient relapsed during the follow-up.Also,21 cases received maintenance therapy including cyclosporine A(n=18),azathioprine(n=2)and mycophenolate(n=1).Conclusions The immunofluorescence IgG subclass in glomeruli and distribution of serum anti-phospholipase A2 receptor in adolescent IMN patients are similar to those in older IMN patients.IMN patients in adolescents responded well to immunosuppressive therapy.Cyclosporine A in low dose as maintenance therapy is effective after achieving remission,and will not increase risk of nephrotoxicity. 目的 明确青少年特发性膜性肾病(IMN)的临床病理特点以及肾脏预后。方法 回顾性研究2012年6月至2014年12月肾活检病理诊断IMN的青少年患者(定义发病年龄在13~24岁),排除膜性肾病继发病因,收集相关的临床、病理资料。随机选择同期年龄大于24岁的IMN患者33例作为对照组。结果 青少年IMN组33人,肾活检时年龄(20±3)岁,男/女比22/11;符合肾病综合征23例,收缩压(127±13)mmHg(1 mmHg=0.133 kPa),舒张压(77±9)mmHg,24小时尿蛋白中位数 5.14(3.39,9.31)g/d,血肌酐62(52,73)μmol/L,血清抗磷脂酶A2受体抗体阳性率54%。与对照组相比,高血压发生率和基线估算肾小球滤过率差异有统计学意义[15.2% 比 39.3%,χ2=4.889,P=0.03;125 ml/(min·1.73m2)比 100 ml/(min·1.73m2),U=137.5,P<0.001]。膜性肾病分期:Ⅰ期1例、Ⅱ期21例、Ⅲ期及以上11例;免疫荧光肾小球IgG亚型阳性率分别为:IgG1 46.9%、IgG2 3.1%、IgG3 56.3%、IgG4 87.5%。与对照组相比,青少年组的肾间质纤维化和肾小动脉病变发生率更低(6.1% 比66.7%,χ2=26.19,P<0.001;15.2% 比 66.7%,χ2=18.11,P<0.001)。青少年组出院后失访3例,其余30例初始方案选择:小剂量泼尼松联合环孢素方案20例、泼尼松联合环磷酰胺7例、泼尼松+霉酚酸酯1例、泼尼松单药2例;中位随访时间18(12,24)个月,随访终点24小时尿蛋白中位数0.20(0.10,0.42)g/d,血肌酐69(56.8,81.3)μmol/L,估算肾小球滤过率(117.3±19.2)ml/(min·1.73m2)。完全缓解17例(56.7%),部分缓解13例(43.4%),完全缓解及部分缓解中位缓解时间分别为3个月和6个月,复发1例。30例随访患者中,完全缓解、部分缓解患者停用免疫抑制剂分别5、4例,其余21例维持治疗:环孢素18例、硫唑嘌呤2例、霉酚酸酯1例。结论 青少年IMN肾小球免疫荧光IgG亚型分布及血清抗磷脂酶A2受体抗体阳性率与对照组IMN相似;免疫抑制治疗对青少年IMN有效,小剂量环孢素作为IMN缓解后的维持性治疗有效且不增加肾毒性发生风险。.[Abstract] [Full Text] [Related] [New Search]
