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与 Kimura 病相关的同时性肾肾小球和间质性病变。

Concurrent Kidney Glomerular and Interstitial Lesions Associated with Kimura's Disease.

机构信息

Department of Pathology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.

Department of Pathology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China,

出版信息

Nephron. 2019;143(2):92-99. doi: 10.1159/000501638. Epub 2019 Aug 6.

Abstract

BACKGROUND

Kimura disease (KD) is a chronic benign inflammatory disorder that is usually manifested as a deep, subcutaneous mass with or without regional lymphadenopathy in the head and neck region. Various types of glomerulonephritis are associated with KD, including minimal change disease (MCD), membranous glomerulopathy (MN), and immunoglobulin (Ig)A-nephropathy. Kidney interstitial lesion associated with KD is rarely reported. The aim of this study was to expand the spectrum of kidney lesions associated with KD.

METHODS

From 2007 to 2016, 12 cases of KD with kidney lesions were retrospectively reviewed. Pathological examinations included hematoxylin and eosin staining, periodic acid-schiff staining, periodic acid-methenamine silver staining, and Masson staining, immunofluorescence, and electron microscope analyses.

RESULTS

Anatomic sites of subcutaneous involvement included head and neck area, arm, and groin. Most cases had elevated IgE level and peripheral eosinophilia. Nephrotic syndrome was the most common kidney manifestation. Pathological results showed 3 types of glomerulonephritis, including 9 cases of MCD, 2 cases of IgA nephropathy, and 1 case of MN. Of note, 4 MCD cases showed concurrent glomerular and interstitial lesions associated with KD, including 2 distinct patterns: (1) a diffusely eosinophilic and lymphatic infiltration similar to KD lesion developed elsewhere and (2) exclusively eosinophilic infiltration in the interstitium. Eight out of 10 patients were responsive to steroid treatment and had complete remission of proteinuria and recovery of kidney function.

CONCLUSION

Our report suggested that, in addition to glomerulonephritis, interstitial lesions may also be associated with KD. Pathologist should pay special attention to differentiated diagnosis when such pathological changes are identified in patients with subcutaneous mass or cervical lymphadenopathy with concurrent kidney manifestation.

摘要

背景

木村病(KD)是一种慢性良性炎症性疾病,通常表现为头颈部深在的皮下肿块,伴有或不伴有区域性淋巴结病。各种类型的肾小球肾炎与 KD 相关,包括微小病变病(MCD)、膜性肾小球病(MN)和免疫球蛋白(Ig)A 肾病。KD 相关的肾间质病变很少有报道。本研究旨在扩大 KD 相关肾病变谱。

方法

回顾性分析 2007 年至 2016 年 12 例 KD 合并肾损害患者。病理检查包括苏木精和伊红染色、过碘酸-雪夫染色、过碘酸-六亚甲基四胺银染色和 Masson 染色、免疫荧光和电子显微镜分析。

结果

皮下受累的解剖部位包括头颈部、手臂和腹股沟。大多数患者有升高的 IgE 水平和外周血嗜酸性粒细胞增多。肾病综合征是最常见的肾脏表现。病理结果显示 3 种类型的肾小球肾炎,包括 9 例 MCD、2 例 IgA 肾病和 1 例 MN。值得注意的是,4 例 MCD 病例同时出现与 KD 相关的肾小球和间质病变,包括 2 种不同的模式:(1)弥漫性嗜酸性粒细胞和淋巴浸润,类似于其他部位的 KD 病变;(2)间质内仅嗜酸性粒细胞浸润。10 例患者中有 8 例对类固醇治疗有反应,蛋白尿完全缓解,肾功能恢复。

结论

本报告提示,除肾小球肾炎外,间质病变也可能与 KD 相关。当病理检查发现皮下肿块或颈淋巴结病伴有肾脏表现的患者存在这些病理改变时,病理医生应特别注意鉴别诊断。

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