Department of Renal Medicine, Northern Care Alliance NHS Foundation Trust, Salford M6 8HD, UK.
Renal Research Laboratory, Kolling Institute of Medical Research, Royal North Shore Hospital, The University of Sydney, Sydney, NSW 2065, Australia.
Genes (Basel). 2023 Jun 26;14(7):1343. doi: 10.3390/genes14071343.
Membranous nephropathy (MN) is the most prevalent cause of nephrotic syndrome amongst the non-diabetic adult population. A fifth of idiopathic nephrotic syndrome cases can be attributed to MN, rising to more than 40% in older patients over 60 years. Most MN cases are classified as being of a primary cause, where there is absence of a secondary disease process explaining its manifestation. Traditionally, the standard approach of diagnosing MN involves performing a kidney biopsy as histological evaluation offers not only conclusive evidence of the diagnosis but also provides valuable information regarding disease chronicity and the presence of any other kidney histopathological features. Nevertheless, kidney biopsy is an invasive procedure which poses risks for the patient including bleeding and pain and bears greater costs for the health system. The identification of the phospholipase A2 receptor (PLA2R) antigen in 2009 was a landmark discovery, one which has evolved our understanding of the disease processes in MN and subsequently our management approach of this condition. Antibodies against PLA2R (PLA2RAb) have since emerged as an attractive non-invasive test option to be applied for the diagnosis and prognostication of primary MN. However, much debate and unknowns remain about the accuracy and reliability of testing for PLA2RAb across various primary MN scenarios. We provide a review summarizing the historical journey of PLA2R in relation to its significance in primary MN and, more importantly, evidence emerging over the years which contemplated the role of PLA2RAb as a diagnostic and prognostic tool in primary MN.
膜性肾病 (MN) 是非糖尿病成年人群中肾病综合征最常见的病因。特发性肾病综合征病例中有五分之一可归因于 MN，在 60 岁以上的老年患者中，这一比例上升至 40%以上。大多数 MN 病例被归类为原发性病因，即没有继发疾病过程来解释其表现。传统上，诊断 MN 的标准方法是进行肾活检，因为组织学评估不仅提供了明确的诊断证据，还提供了有关疾病慢性和任何其他肾脏组织病理学特征的有价值信息。然而，肾活检是一种有创性的程序，会给患者带来包括出血和疼痛在内的风险，并且对医疗系统的成本更高。2009 年发现磷脂酶 A2 受体 (PLA2R) 抗原是一个具有里程碑意义的发现，它改变了我们对 MN 疾病过程的理解，并随后改变了我们对这种疾病的管理方法。针对 PLA2R 的抗体 (PLA2RAb) 作为一种有吸引力的非侵入性检测选择，已被应用于原发性 MN 的诊断和预后。然而，关于 PLA2RAb 在各种原发性 MN 情况下的准确性和可靠性仍然存在很多争议和未知数。我们提供了一篇综述，总结了 PLA2R 在原发性 MN 中的历史发展历程及其重要意义，更重要的是，总结了多年来 PLA2RAb 作为原发性 MN 诊断和预后工具的作用的相关证据。
