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

发布时间：2018-11-08 08:20

【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。
[Abstract]:Objective: to analyze the clinicopathological features, treatment and prognosis of idiopathic eosinophil syndrome (IHES) with renal damage. Methods: the clinical manifestations of IHES patients with renal damage were retrospectively analyzed, and the clinical, pathological, therapeutic and prognostic features of renal damage were discussed. Results: 18 patients, 13 males and 5 females, had a median age of 36 years (967 years). The clinical manifestations were multi-system involvement with kidney involvement, followed by skin (27.8%), lung (27.8%), gastrointestinal tract (27.8%) and nerve (11.1%). The eosinophils in blood were significantly increased (1 670 0 15 100 / 渭 l). Renal damage was found in 12 cases (66.7%) with nephrotic syndrome, 4 cases (22.2%) with abnormal urine examination, 2 cases (11.1%) with acute renal injury, 6 cases (33.3%) with chronic renal insufficiency, 15 cases with renal biopsy. There were 3 cases of membranous hyperplasia, 3 cases of podocytosis, 2 cases of Mesangial hyperplasia, 2 cases of IgA nephropathy, 2 cases of membranous nephropathy, 2 cases of chronic interstitial nephritis and 1 case of focal segmental sclerosis. Eosinophilic granulocyte infiltration was found in renal interstitium in 11 cases and eosinophil infiltration in glomeruli in 3 cases. Immunofluorescence examination showed immunoglobulin and complement deposition in 12 cases. Serum eosinophil decreased after treatment with glucocorticoid or immunosuppressant. Of the 15 cases, 14 cases had urinary protein decrease or negative, renal function recovered or stabilized, 8 cases increased eosinophils, 1 case urine protein increased, 1 case progressed to end-stage nephropathy after steroid withdrawal. Conclusion: renal damage may occur in IHES, with nephrotic syndrome as the main manifestation, with various pathological types and eosinophil infiltration in most of renal stroma. The prognosis of most patients was better after hormone therapy.
【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;
【基金】：国家自然科学基金(81270799) 国家科技支撑计划课题(2015BAI12B05,2015BAI12B02) 江苏省临床医学科技专项(BL2012007)
【分类号】：R597;R692

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