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首页> 中文期刊> 《肾脏病与透析肾移植杂志》 >特发性嗜酸性粒细胞增多综合征肾脏损害的临床病理分析

特发性嗜酸性粒细胞增多综合征肾脏损害的临床病理分析

         
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摘要

目的:分析特发性嗜酸性粒细胞增多综合征(IHES)伴肾脏损害患者的临床病理特征、治疗及预后.方法:回顾性分析IHES肾脏损害患者的临床表现,探讨其肾脏损害的临床、病理、治疗及预后特点. 结果:18例患者,男性13例、女性5例,发病中位年龄36岁(9 ～ 67岁).临床表现为多系统受累,均有肾脏受累,其次是皮肤(27.8％)、肺脏(27.8％)、胃肠道(27.8％)、神经(11.1％).血嗜酸粒细胞均明显升高(1 670～ 15 100/μl).肾损害表现为肾病综合征12例(66.7％)、尿检异常4例(22.2％)、急性肾损伤2例(11.1％)、慢性肾功能不全共6例(33.3％).15例行肾活检,病理表现为膜增生样病变3例、足细胞病3例、系膜增生性病变2例、IgA肾病2例、膜性肾病2例、慢性间质性肾炎2例、局灶节段性硬化症1例;11例肾间质见嗜酸性粒细胞浸润,3例肾小球见嗜酸性粒细胞浸润;免疫荧光检查:12例见免疫球蛋白、补体沉积.应用糖皮质激素或联合免疫抑制剂治疗后血嗜酸粒细胞均下降;15例随访患者中,14例尿蛋白下降或转阴、肾功能恢复或稳定,停激素后8例嗜酸性粒细胞增加,1例尿蛋白增加,1例进展至终末期肾病. 结论:IHES可发生肾脏损害,以肾病综合征为主要表现,病理类型多种多样,肾间质多数可见嗜酸性粒细胞浸润.多数患者经激素治疗后预后较好.%Objective:To explore the clinico-pathologic features,treatment and prognosis of renal impairment in patients with idiopathic hypereosinophilic syndrome (IHES).Methodology:Eighteen patients with IHES were enrolled into this retrospective study.Their clinical,pathologic and follow-up data were investigated.Results:They were 13 male and 8 female with a median age of 36 years old (ranged from 9 to 67 y).They presented multiple organ dysfunctions including kidney (100％),skin (27.8％),lung (27.8％),gastrointestinal tract (27.8％) and peripheral nerve involvement (11.1％).The eosinophil count significantly inc reased (1 670～15 100/μl).Renal impairment was included nephrotic syndrome in 12 cases (66.7％),urinary ahnormalities in 4 (22.2％),acute kidney injury in 2 (11.1％),and chronic renal insufficiency in 6 (33.3％).15 cases underwent renal biopsy,the histological diagnosis was membranoproliferative glomerulonephritis in 3 cases,podocyte disease in 3,mesangial proliferative glomerulonephritis in 2,IgA nephropathy in 2,membranous nephropathy in 2,chronic interstitial nephritis in 2,and focal segmental sclerosis in one case.Eosinophil infiltrated into renal interstitium in 11 and into Glomerulus in 3) cases.Immunoglobulin or complement deposited in glomerulus in 12 cases.After treatment of glucocorticoid only or combination with other immunosuppressant,the eosinophil count was decreased.15 patients were followed-up,14(93.3％) showed the urinary protein decreased or renal function recovered.When the glucocorticoid is discontinued,eosinophil increased (8 cases),urine protein increased (1) in some patients,even progression to end-stage renal disease (1).Conclusion:IHES can induce renal impairmen.Nephrotic syndrome were common as clinical features.Pathologic types were various,infiltration of eosinophil were the common pathologic features.Glucocorticoid can improve the renal impairmeuts.Prognosis were good in most of the patients.

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  • 来源
    《肾脏病与透析肾移植杂志》 |2017年第4期|301-306|共6页
  • 作者

    董建华; 许书添; 涂义姚; 郭锦洲; 周玉超; 李世军;

  • 作者单位

    南京总医院肾脏科国家肾脏疾病临床医学研究中心 全军肾脏病研究所 南京,210016;

    南京总医院肾脏科国家肾脏疾病临床医学研究中心 全军肾脏病研究所 南京,210016;

    南京总医院肾脏科国家肾脏疾病临床医学研究中心 全军肾脏病研究所 南京,210016;

    南京总医院肾脏科国家肾脏疾病临床医学研究中心 全军肾脏病研究所 南京,210016;

    南京总医院肾脏科国家肾脏疾病临床医学研究中心 全军肾脏病研究所 南京,210016;

    南京总医院肾脏科国家肾脏疾病临床医学研究中心 全军肾脏病研究所 南京,210016;

  • 原文格式 PDF
  • 正文语种 chi
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  • 关键词

    嗜酸性粒细胞增多综合征; 特发性; 临床病理特征; 糖皮质激素;

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