Review of Glomerular Diseases: Focal Segmental Glomerulosclerosis (FSGS) and Minimal Change Disease (MCD)

Gursharan Sohi
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Abstract

Purpose: Idiopathic focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) are chronic glomerulopathies which may compromise patients’ quality of life, and for which there is no cure. This literature review aimed to summarize our current understanding of the pathophysiology, clinical characteristics, and best available treatment for the two conditions in order to outline a consolidated treatment protocol and identify future research considerations. Methods: PubMed was systematically searched by a single reviewer in order to identify primary studies pertaining to the diagnosis, treatment and classification of FSGS and MCD. Additionally, a hand search of UpToDate was conducted to glean further information about the best available evidence as summarized for clinician use. Relevant information was extracted and synthesized. Results: Primary FSGS and MCD result from distinct pathogenic mechanisms, hypothesized to involve kidney injury via immune dysregulation. Patients require a kidney biopsy for diagnostic purposes. First-line treatment involves glucocorticoids (i.e. prednisone), although patients’ responsiveness may be inconsistent; second-line treatment is immunotherapy. Conclusion: This review summarized clinically-important information about FSGS and MCD, and emphasized the need for further research in the field of clinical nephrology. Large scale trials such as the Cure Glomerulonephropathy should be conducted to gather information about the affected population.
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肾小球疾病综述:局灶节段性肾小球硬化(FSGS)和微小改变病(MCD)
目的:特发性局灶节段性肾小球硬化(FSGS)和微小改变病(MCD)是一种慢性肾小球疾病,可能会影响患者的生活质量,并且无法治愈。本文献综述旨在总结我们目前对这两种疾病的病理生理学、临床特征和最佳治疗方法的理解,以概述统一的治疗方案并确定未来的研究考虑。方法:由一名审稿人系统地检索PubMed,以确定与FSGS和MCD的诊断、治疗和分类有关的初步研究。此外,进行了UpToDate的手动搜索,以收集有关临床医生使用的最佳现有证据的进一步信息。提取并综合相关信息。结果:原发性FSGS和MCD有不同的致病机制,假设与免疫失调引起的肾损伤有关。患者需要进行肾脏活检以进行诊断。一线治疗包括糖皮质激素(即强的松),尽管患者的反应性可能不一致;二线治疗是免疫疗法。结论:本文综述了FSGS和MCD的临床重要信息,并强调了在临床肾脏学领域进一步研究的必要性。应该进行诸如“治愈肾小球肾病”这样的大规模试验,以收集有关受影响人群的信息。
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