Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, Innsbruck, Austria.
Department of Pediatrics, Medical University of Innsbruck, Innsbruck, Austria.
Front Immunol. 2019 Jul 17;10:1669. doi: 10.3389/fimmu.2019.01669. eCollection 2019.
A major obstacle in kidney transplantation for primary focal segmental glomerulosclerosis (FSGS) is the risk of disease recurrence. Recurrent FSGS affects up to 60% of first kidney grafts and exceeds 80% in patients who have lost their first graft due to recurrent FSGS. Clinical and experimental evidence support the hypothesis that a circulating permeability factor is the mediator in the pathogenesis of primary and recurrent disease. Despite all efforts, the causing agent has not yet been identified. Several treatment options for the management of recurrent FSGS have been proposed. In addition to plasma exchange, B-cell depleting antibodies are effective in recurrent FSGS. This indicates, that the secretion and/or activity of the postulated circulating permeability factor(s) may be B-cell related. This review summarizes the current knowledge on permeability factor(s) possibly related to the disease and discusses strategies for the management of recurrent FSGS. These include profound B-cell depletion prior to transplantation, as well as the salvage of an allograft affected by recurrent FSGS by transfer into a second recipient.
在原发性局灶节段性肾小球硬化症(FSGS)的肾移植中,一个主要障碍是疾病复发的风险。复发的 FSGS 影响多达 60%的首次肾移植,而在因复发 FSGS 而失去首次移植的患者中,这一比例超过 80%。临床和实验证据支持这样一种假设,即循环通透性因子是原发性和复发性疾病发病机制中的介质。尽管做出了各种努力,但仍未确定致病因子。已经提出了几种治疗复发性 FSGS 的选择方案。除了血浆置换,B 细胞耗竭抗体在复发性 FSGS 中也有效。这表明,所假设的循环通透性因子的分泌和/或活性可能与 B 细胞有关。这篇综述总结了与疾病相关的通透性因子的最新知识,并讨论了复发性 FSGS 的管理策略。这些策略包括在移植前进行深度 B 细胞耗竭,以及通过将受复发性 FSGS 影响的同种异体移植物转移到第二个受者中来挽救同种异体移植物。