Expert Discussion on Immune Complex-Mediated Membranoproliferative Glomerulonephritis: Challenges and Considerations

IF 3.4 3区 医学 Q2 MEDICINE, RESEARCH & EXPERIMENTAL
Richard A. Lafayette, Vivek Charu, Richard J. Glassock
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Abstract

Immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) is a rare pattern of kidney injury and a progressive nephropathy characterized by the glomerular deposition of immune complexes and complement proteins. The IC-MPGN pattern of injury exhibits a membranoproliferative glomerulonephritis appearance by light microscopy and occurs secondary to various conditions or, more rarely, idiopathically, when no underlying etiology can be determined. Kidney biopsy is the only method for identifying IC-MPGN, distinguishing between IC-MPGN and complement 3 glomerulopathy (C3G), and for providing critical pathologic insights that guide further clinical evaluation for underlying etiologies and inform patient management. Given the progressive nature of IC-MPGN, it is crucial to identify patients early and to define the underlying pathophysiology for timely and appropriate treatment. However, several challenges remain in the accurate interpretation of kidney biopsy specimens and the effective treatment of idiopathic disease. In this commentary, two nephrologists and a nephropathologist review best practices in the clinical and histopathologic evaluation of IC-MPGN and discuss the central role of kidney biopsy in the differentiation of IC-MPGN and C3G. The challenges and considerations discussed are explored through an illustrative case of idiopathic disease, drawn from the authors' clinical experiences. Finally, remaining unmet needs are highlighted, and future perspectives on targeted treatments under investigation for patients with idiopathic IC-MPGN are provided.

专家讨论免疫复合物介导的膜增生性肾小球肾炎:挑战和考虑。
免疫复合物介导的膜增殖性肾小球肾炎(IC-MPGN)是一种罕见的肾损伤和进行性肾病,其特征是免疫复合物和补体蛋白的肾小球沉积。损伤的IC-MPGN模式在光镜下表现为膜增生性肾小球肾炎,继发于各种疾病,或者更罕见的是,在无法确定潜在病因的情况下,是特发性的。肾活检是识别IC-MPGN、区分IC-MPGN和补体3型肾小球病(C3G)的唯一方法,并提供关键的病理见解,指导进一步的临床评估潜在病因,并为患者管理提供信息。鉴于IC-MPGN的进行性,早期识别患者并确定潜在的病理生理学以及时和适当的治疗是至关重要的。然而,在肾脏活检标本的准确解释和特发性疾病的有效治疗方面仍然存在一些挑战。在这篇评论中,两位肾病学家和一位肾病病理学家回顾了IC-MPGN的临床和组织病理学评估的最佳实践,并讨论了肾活检在IC-MPGN和C3G鉴别中的核心作用。所讨论的挑战和考虑是通过一个说明性的特发性疾病的情况下,从作者的临床经验得出的探讨。最后,强调了尚未满足的需求,并提供了正在研究的特发性IC-MPGN患者靶向治疗的未来前景。
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来源期刊
Advances in Therapy
Advances in Therapy 医学-药学
CiteScore
7.20
自引率
2.60%
发文量
353
审稿时长
6-12 weeks
期刊介绍: Advances in Therapy is an international, peer reviewed, rapid-publication (peer review in 2 weeks, published 3–4 weeks from acceptance) journal dedicated to the publication of high-quality clinical (all phases), observational, real-world, and health outcomes research around the discovery, development, and use of therapeutics and interventions (including devices) across all therapeutic areas. Studies relating to diagnostics and diagnosis, pharmacoeconomics, public health, epidemiology, quality of life, and patient care, management, and education are also encouraged. The journal is of interest to a broad audience of healthcare professionals and publishes original research, reviews, communications and letters. The journal is read by a global audience and receives submissions from all over the world. Advances in Therapy will consider all scientifically sound research be it positive, confirmatory or negative data. Submissions are welcomed whether they relate to an international and/or a country-specific audience, something that is crucially important when researchers are trying to target more specific patient populations. This inclusive approach allows the journal to assist in the dissemination of all scientifically and ethically sound research.
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