Table of Contents Author Guidelines Submit a Manuscript
BioMed Research International
Volume 2017 (2017), Article ID 1936372, 14 pages
https://doi.org/10.1155/2017/1936372
Review Article

Recent Progress in Deciphering the Etiopathogenesis of Primary Membranous Nephropathy

1Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria
2Pediatric Nephrology, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany
3Department of Nephrology, UZ Leuven, Leuven, Belgium
4Department of Immunology and Microbiology, KU Leuven, Leuven, Belgium
5Department of Pediatrics, Yonsei University College of Medicine, Severance Children’s Hospital, Seoul, Republic of Korea

Correspondence should be addressed to Andreas Kronbichler

Received 27 February 2017; Accepted 18 July 2017; Published 17 August 2017

Academic Editor: Christophe Duranton

Copyright © 2017 Andreas Kronbichler et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Primary membranous nephropathy (MN) is the leading cause of nephrotic syndrome in adults. Discovery of several antibodies has contributed to an increased understanding of MN. Antibodies against the M-type phospholipase A2 receptor (PLA2R) are present in 50–100% with primary MN and are associated with a lower frequency of spontaneous remission. High levels are linked with a higher probability of treatment resistance, higher proteinuria, and impaired renal function, as well as a more rapid decline of kidney function during follow-up. Immunologic remission precedes reduction of proteinuria by months. Pretransplant evaluation of PLA2R antibodies is warranted to predict recurrence of disease following renal transplantation. Several risk alleles related to the PLA2R1 gene and within the HLA loci have been identified, whereas epitope spreading of PLA2R may predict treatment response. More recently, thrombospondin type 1 domain-containing 7A (THSD7A) antibodies have been discovered in primary MN. Several other rare antigens have been described, including antibodies against neutral endopeptidase as a cause of antenatal MN and circulating cationic bovine serum albumin as an antigen with implications in childhood MN. This review focuses on the progress with a special focus on diagnostic accuracy, predictive value, and treatment implications of the established and proposed antigens.