Hoxha Elion, von Haxthausen Franziska, Wiech Thorsten, Stahl Rolf A K
III. Medizinische Klinik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany.
SFB 1192, Universitätsklinikum Hamburg-Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany.
Pflugers Arch. 2017 Aug;469(7-8):989-996. doi: 10.1007/s00424-017-2000-4. Epub 2017 May 29.
Since the discovery of the phospholipase A receptor 1 (PLAR1) and thrombospondin type-1 domain-containing 7A (THSD7A) as endogenous antigens involved in the development of membranous nephropathy (MN) in over 80% of adult patients, substantial progress in the diagnosis, prognosis, and therapy of MN has been made. In most cases of patients with MN, it is now possible to specifically define the responsible pathogenic mechanisms of disease and make a diagnosis even without a renal biopsy. Moreover, the presence of antibodies in the blood and the detection of the antigens in renal biopsies allow the definite diagnosis without the morphologic uncertainties, which now still apply for only about 20% of all renal biopsies showing MN. The discovery that the expression of THSD7A in malignant tumors might serve as the site of primary antigen recognition for the immune system to start MN might lead to a better understanding of not only tumor-associated MN, which accounts for up to 10% of all patients with MN, but also of the pathomechanisms relevant for MN development in general.
自从发现磷脂酶A受体1（PLAR1）和含血小板反应蛋白1型结构域7A（THSD7A）作为超过80%成年患者膜性肾病（MN）发病过程中的内源性抗原以来，MN在诊断、预后和治疗方面取得了重大进展。在大多数MN患者中，现在即使不进行肾活检也能够明确界定疾病的致病机制并做出诊断。此外，血液中抗体的存在以及肾活检中抗原的检测使得诊断明确，避免了形态学上的不确定性，而目前这种不确定性仅适用于约20%所有显示MN的肾活检。THSD7A在恶性肿瘤中的表达可能作为免疫系统引发MN的主要抗原识别位点这一发现，不仅可能有助于更好地理解占所有MN患者多达10%的肿瘤相关性MN，还可能有助于更好地理解MN发生发展的一般病理机制。
