狼疮性肾炎:电子显微镜的重要贡献。

Luan Truong, Surya V Seshan
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摘要

背景:系统性红斑狼疮(SLE)是一种多系统自身免疫性疾病的主要原型,由细胞和抗体介导的机制共同参与,导致严重的肾脏损害。肾活检诊断是SLE临床肾脏疾病的金标准,它包括广泛的适应症。总结:SLE患者的肾脏疾病可累及肾小球、小管间质和/或血管间室,它们之间并不相互排斥。在大多数情况下,基本的发病机制涉及免疫复合物的组织沉积和/或细胞介导的机制,通过光镜、免疫组织化学方法和电子显微镜(EM)鉴定,引起肾小球内增生、炎症和其他组织反应。这些会产生一系列的组织学病变,这取决于广泛的临床触发因素的参与,即遗传、血清学和免疫因素,与它们潜在的发病潜力相关。除了光镜和免疫荧光显微镜外,在这种情况下,EM有助于准确诊断,评估疾病活动性,描述亚类,与原发性非狼疮肾病变区分,识别有组织的沉积物,很少识别其他形式的非免疫性复杂病变,如足细胞病变,淀粉样变性和血栓性微血管病变。主要信息:SLE中大多数肾脏病变的独特电镜表现包括免疫复合物和非免疫复合物疾病以及重叠实体。常规超微结构检查不仅为狼疮患者的初次和重复肾活检提供了重要的诊断和预后信息,而且有助于了解疾病过程的潜在病理生理学。
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Lupus Nephritis: The Significant Contribution of Electron Microscopy.

Background: Systemic lupus erythematosus (SLE) represents a principal prototype of a multisystemic autoimmune disease with the participation of both cell- and antibody-mediated mechanisms causing significant renal impairment. A renal biopsy diagnosis is the gold standard for clinical renal disease in SLE, which includes a broad range of indications.

Summary: Renal disease in SLE can involve glomerular, tubulointerstitial, and/or vascular compartments, none of which are mutually exclusive. In most instances, the basic pathogenetic mechanism involves tissue deposition of immune complexes and/or cell-mediated mechanisms, identified by light microscopy, immunohistochemical methods, and electron microscopy (EM), evoking intraglomerular proliferative, inflammatory, and other tissue responses. These produce a spectrum of histologic lesions, depending on the participation of a wide range of clinical triggers, namely, genetic, serological, and immunological factors, correlating with their underlying pathogenetic potential. In addition to light and immunofluorescence microscopy, EM in this setting facilitates an accurate diagnosis, assesses disease activity, delineates subclasses, differentiates from primary forms of non-lupus renal lesions, identifies organized deposits, and rarely, identifies other forms of nonimmune complex lesions such as podocytopathies, amyloidosis, and thrombotic microangiopathy.

Key messages: EM findings that are distinctive for most of the renal lesions in SLE include immune complex and nonimmune complex diseases as well as overlapping entities. Routine ultrastructural examination not only provides significant diagnostic and prognostic information from both initial and repeat renal biopsies from lupus patients but also contributes toward the understanding of the underlying pathophysiology of the disease process.

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