Shah Monarch, DeLaat Andrew, Cavanaugh Corey
Division of Nephrology, University of Virginia, Charlottesville, VA, United States.
Liberty University College of Osteopathic Medicine, Lynchburg, VA, United States.
Front Nephrol. 2023 Jan 27;3:1110355. doi: 10.3389/fneph.2023.1110355. eCollection 2023.
Primary membranous nephropathy remains one of the most frequent causes of nephrotic syndrome in adults. It is an autoimmune disorder in which auto-antibodies target antigens at the podocytes cell membrane-basement membrane interface. Our understanding of membranous nephropathy has expanded dramatically as of late. After the initial discovery of the phospholipase A2 receptor auto-antibody in 2009, eight more antigens have been discovered. These discoveries have led to refinement in our understanding of the pathogenesis, diagnosis, and natural history of primary membranous nephropathy. Now, many experts advocate for redefining primary membranous nephropathy based on antigen, potentially shedding the primary and secondary nomenclature. Recently, therapies for primary membranous have also expanded. Immunosuppressive therapies like cyclophosphamide and rituximab, which primarily target B-cells, remain the cornerstone of therapy. However, there is still significant room for improvement, as many as 30-40% do not respond to this therapy according to recent trials. Additionally, drugs targeting complement, and other novel therapies are also under investigation. In this review we will discuss the available therapies for primary membranous nephropathy in light of recent clinic trials like GEMRITUX, MENTOR, RI-CYCLO, and STARMEN, as well as management strategies. While the last 10 years have seen a boom in our mechanistic understanding of this ever-diversifying disease, we are likely to see a similar boom in the therapeutic options in the years to come.
原发性膜性肾病仍然是成人肾病综合征最常见的病因之一。它是一种自身免疫性疾病,自身抗体靶向足细胞细胞膜-基底膜界面的抗原。近年来,我们对膜性肾病的认识有了显著扩展。2009年首次发现磷脂酶A2受体自身抗体后,又发现了另外8种抗原。这些发现使我们对原发性膜性肾病的发病机制、诊断和自然史的认识更加精确。现在,许多专家主张根据抗原重新定义原发性膜性肾病,可能摒弃原发性和继发性的命名法。最近,原发性膜性肾病的治疗方法也有所扩展。以B细胞为主要靶点的免疫抑制疗法,如环磷酰胺和利妥昔单抗,仍然是治疗的基石。然而,仍有很大的改进空间,根据最近的试验,多达30%-40%的患者对这种疗法没有反应。此外,针对补体的药物和其他新型疗法也在研究中。在这篇综述中,我们将根据最近的临床试验,如GEMRITUX、MENTOR、RI-CYCLO和STARMEN,以及管理策略,讨论原发性膜性肾病的现有治疗方法。虽然在过去10年里,我们对这种不断变化的疾病的机制理解有了很大进展,但在未来几年,我们可能会看到治疗选择也有类似的进展。