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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 analyze the clinicopathological characteristics, treatment and prognosis of patients with idiopathic eosinophilia syndrome (IHES) and renal damage. Methods: The clinical manifestations of renal damage in IHES were analyzed retrospectively to investigate the clinical, pathological, therapeutic and prognostic characteristics of renal damage. Results: Eighteen patients, 13 males and 5 females, had a median age of 36 years (range, 9 to 67 years). The clinical manifestations of multiple system involvement, all have kidney involvement, followed by the skin (27.8%), lung (27.8%), gastrointestinal (27.8%), nerve (11.1%). Blood eosinophils were significantly increased (1 670 ~ 15 100 / μl). Kidney damage manifested as nephrotic syndrome in 12 cases (66.7%), urinalysis in 4 cases (22.2%), acute renal injury in 2 cases (11.1%) and chronic renal insufficiency in 6 cases (33.3%). 15 cases of renal biopsy, the pathological manifestations of membranoproliferative lesions in 3 cases, podocyte disease in 3 cases, mesangial proliferative lesions in 2 cases, IgA nephropathy in 2 cases, membranous nephropathy in 2 cases, chronic interstitial nephritis in 2 cases, Bureau 1 case of focal segmental sclerosis; 11 cases of eosinophilic infiltration of renal interstitium, 3 cases of glomerular eosinophil infiltration; Immunofluorescence: immunoglobulin in 12 cases, complement deposition. Eosinophils were decreased after treatment with glucocorticoids or combined immunosuppressive drugs. In the 15 follow-up cases, urinary protein decreased or turned negative in 14 cases, and renal function recovered or stabilized. Eosinophils in 8 patients were increased , 1 case of urinary protein increased, 1 case progressed to end-stage renal disease. CONCLUSIONS: IHES can occur renal damage, with nephrotic syndrome as the main manifestation, a variety of pathological types, most of the renal interstitial eosinophilic infiltration can be seen. The majority of patients with good prognosis after hormone therapy.