【摘要】：目的:分析特發(fā)性嗜酸性粒細胞增多綜合征(IHES)伴腎臟損害患者的臨床病理特征、治療及預后。方法:回顧性分析IHES腎臟損害患者的臨床表現(xiàn),探討其腎臟損害的臨床、病理、治療及預后特點。結(jié)果:18例患者,男性13例、女性5例,發(fā)病中位年齡36歲(9~67歲)。臨床表現(xiàn)為多系統(tǒng)受累,均有腎臟受累,其次是皮膚(27.8%)、肺臟(27.8%)、胃腸道(27.8%)、神經(jīng)(11.1%)。血嗜酸粒細胞均明顯升高(1 670~15 100/μl)。腎損害表現(xiàn)為腎病綜合征12例(66.7%)、尿檢異常4例(22.2%)、急性腎損傷2例(11.1%)、慢性腎功能不全共6例(33.3%)。15例行腎活檢,病理表現(xiàn)為膜增生樣病變3例、足細胞病3例、系膜增生性病變2例、IgA腎病2例、膜性腎病2例、慢性間質(zhì)性腎炎2例、局灶節(jié)段性硬化癥1例;11例腎間質(zhì)見嗜酸性粒細胞浸潤,3例腎小球見嗜酸性粒細胞浸潤;免疫熒光檢查:12例見免疫球蛋白、補體沉積。應(yīng)用糖皮質(zhì)激素或聯(lián)合免疫抑制劑治療后血嗜酸粒細胞均下降;15例隨訪患者中,14例尿蛋白下降或轉(zhuǎn)陰、腎功能恢復或穩(wěn)定,停激素后8例嗜酸性粒細胞增加,1例尿蛋白增加,1例進展至終末期腎病。結(jié)論:IHES可發(fā)生腎臟損害,以腎病綜合征為主要表現(xiàn),病理類型多種多樣,腎間質(zhì)多數(shù)可見嗜酸性粒細胞浸潤。多數(shù)患者經(jīng)激素治療后預后較好。
[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.
【作者單位】： 南京總醫(yī)院腎臟科國家腎臟疾病臨床醫(yī)學研究中心全軍腎臟病研究所;
【基金】：國家自然科學基金(81270799) 國家科技支撐計劃課題(2015BAI12B05,2015BAI12B02) 江蘇省臨床醫(yī)學科技專項(BL2012007)
【分類號】：R597;R692

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