Murtas Corrado, Ghiggeri Gian Marco
Division of Nephrology and Dialysis, Ospedale San Martino, ASL 5, via Rockefeller, 09170, Oristano, Italy.
Division of Nephrology Dialysis and Transplantation, Istituto Giannina Gaslini, Genoa, Italy.
J Nephrol. 2016 Aug;29(4):469-78. doi: 10.1007/s40620-016-0268-7. Epub 2016 Jan 25.
Membranous nephropathy is a frequent cause of nephrotic syndrome in adults. In most patients, it appears as a primary renal disease but in about 20 % of cases membranous nephropathy is associated with systemic conditions such as systemic lupus erythematosus, infections or cancer, or with drug exposure. Reliable differentiation between primary and secondary membranous nephropathy has important implications for the patient, because of different therapeutic approaches between the different forms. The recent in vivo definition of glomerular targets of autoimmunity in idiopathic membranous nephropathy represented a real breakthrough and nowadays more than one podocyte antigen is considered in some way implicated in the pathogenesis of human membranous nephropathy. Specific antibodies against all these components have been detected in serum of patients and could become biomarkers of membranous nephropathy and/or of disease activity. In this brief review, we discuss the usefulness of newly described autoantibodies in the differential diagnosis of secondary membranous nephropathy. Histological clues for recognizing the two pathological entities are also analysed with regard to the available scientific evidence on this issue. Our evaluation shows that more research is needed to identify the best approach to reach a correct diagnosis of primary or secondary membranous nephropathy.
膜性肾病是成人肾病综合征的常见病因。在大多数患者中,它表现为原发性肾脏疾病,但在约20%的病例中,膜性肾病与系统性疾病如系统性红斑狼疮、感染或癌症相关,或与药物暴露有关。原发性和继发性膜性肾病之间的可靠区分对患者具有重要意义,因为不同形式的治疗方法不同。最近对特发性膜性肾病自身免疫性肾小球靶点的体内定义是一个真正的突破,如今,不止一种足细胞抗原在某种程度上被认为与人类膜性肾病的发病机制有关。在患者血清中已检测到针对所有这些成分的特异性抗体,这些抗体可能成为膜性肾病和/或疾病活动的生物标志物。在这篇简短的综述中,我们讨论了新描述的自身抗体在继发性膜性肾病鉴别诊断中的作用。关于识别这两种病理实体的组织学线索,也根据关于这个问题的现有科学证据进行了分析。我们的评估表明,需要更多的研究来确定正确诊断原发性或继发性膜性肾病的最佳方法。