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中华临床医师杂志(电子版) ›› 2024, Vol. 18 ›› Issue (04) : 397 -401. doi: 10.3877/cma.j.issn.1674-0785.2024.04.011

综述

结节病肾损害的研究进展
王倩1, 李敏1, 白璐1, 王保兴1, 李英1,()   
  1. 1. 050000 石家庄,河北医科大学第三医院肾内科 河北省肾脏病研究中心
  • 收稿日期:2024-01-16 出版日期:2024-04-15
  • 通信作者: 李英
  • 基金资助:
    国家自然科学基金资助项目(82070743); 河北省卫生健康委科研计划项目(20221164)

Progress in research of renal sarcoidosis

Qian Wang1, Min Li1, Lu Bai1, Baoxing Wang1, Ying Li1,()   

  1. 1. Department of Nephrology, The Third Hospital of Hebei Medical University, Hebei Nephrology Research Center, Shijiazhuang 050051, China
  • Received:2024-01-16 Published:2024-04-15
  • Corresponding author: Ying Li
引用本文:

王倩, 李敏, 白璐, 王保兴, 李英. 结节病肾损害的研究进展[J]. 中华临床医师杂志(电子版), 2024, 18(04): 397-401.

Qian Wang, Min Li, Lu Bai, Baoxing Wang, Ying Li. Progress in research of renal sarcoidosis[J]. Chinese Journal of Clinicians(Electronic Edition), 2024, 18(04): 397-401.

结节病(sarcoidosis)是一种病因和发病机制不明的系统性肉芽肿性疾病,可累及肺、淋巴结、皮肤、肾、眼等多个脏器,其中肾脏受累发生率较高。结节病肾损害(RS)的主要临床表现为肾小管功能障碍、高钙血症相关性肾脏疾病;病理学特征是非干酪坏死性上皮细胞样肉芽肿的积聚、间质性肾炎的形成。结节病肾损害起病隐匿,临床表现无特异性,诊断缺乏统一标准,易被临床医师忽视,严重肾脏受累者可引起急、慢性肾衰竭,甚至进展至终末期肾脏病。早期诊断和及时应用糖皮质激素或其他免疫抑制剂治疗可改善预后。本文就结节病肾损害的流行病学、发病机制、临床表现、治疗及预后做一综述,为结节病肾损害临床管理提供新的思路。

Sarcoidosis is a systemic granulomatous disease with unknown etiology and pathogenesis, which can affect multiple organs such as the lungs, lymph nodes, skin, kidneys, and eyes, among which the incidence of kidney involvement is relatively high. The main clinical manifestations of renal sarcoidosis are renal tubular dysfunction and hypercalcemia related renal disease. The pathological characteristics are accumulation of noncaseating necrotizing epithelioid granuloma and interstitial nephritis. Since renal sarcoidosis has an insidious onset and non-specific clinical manifestations and their is a lack of unified diagnostic standards, it is easy to be ignored by clinicians. Severe kidney involvement can lead to acute or chronic renal failure, and even progress to end-stage renal disease. Early diagnosis and timely application of glucocorticoids or immunosuppressants can improve the prognosis. This article reviews the epidemiology, pathogenesis, clinical manifestations, treatment, and prognosis of renal sarcoidosis, with an aim to provide a new idea for clinical management of renal sarcoidosis.

图1 非干酪样肉芽肿及间质性肾炎形成机制 注:TLR为Toll样受体;NF-κB为核因子激酶B;AP1为激活蛋白1;NLR为NOD样受体;APC为抗原呈递细胞;DC为树突状细胞;MC为巨噬细胞;IFN-γ为干扰素-γ;IL为白细胞介素;PI-3K为磷脂酰肌醇3激酶;mTORC1为雷帕霉素复合物1的哺乳动物靶点;Treg为调节T细胞;Th1为辅助T细胞1;Th17为辅助T细胞17
图2 结节病肾损害特征性病理表现。图a为非干酪样肉芽肿形成(PASM×200);图b为非干酪样肉芽肿形成(H&E×200);图c为部分肾小管上皮细胞崩解、脱落及再生,基底膜裸露,肾小管损伤(PASM×200);图d为肾间质单核细胞和淋巴细胞浸润(H&E×200) 注:病理图片由河北医科大学第三医院肾脏病实验室提供
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