Article Text

PDF
Primary membranous nephropathy: comprehensive review and historical perspective
  1. Krishna C Keri1,
  2. Samuel Blumenthal1,
  3. Varsha Kulkarni2,
  4. Laurence Beck3,
  5. Tepsiri Chongkrairatanakul1
  1. 1 Division of Nephrology, Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
  2. 2 Department of Pediatrics, University of Alabama, Birmingham, Alabama, USA
  3. 3 Renal Section, Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, USA
  1. Correspondence to Dr Krishna C Keri, Division of Nephrology, Department of Medicine, Medical College of Wisconsin, Milwaukee, WI 53226, USA; krishnakeri{at}gmail.com

Abstract

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in non-diabetic Caucasian adults over 40 years of age. It has an estimated incidence of 8–10 cases per 1 million. Fifty per cent of patients diagnosed with primary MN continue to have nephrotic syndrome and 30% of patients may progress to end-stage renal disease over 10 years. Although it was recognised as a distinct clinic-pathological entity in 1940s by immunofluorescence and electron microscopy, the pathogenesis and treatment have become more apparent only in the last decade. Discovery of M-type phospholipase A2 receptor (PLA2R) antibodies and thrombospondin type 1 domain-containing 7A antibodies has given new perspectives in understanding the pathogenesis of the disease process. Anti-PLA2R antibody is the first serologic marker that has promising evidence to be used as a tool to prognosticate the course of the disease. More importantly, therapeutic agents such as rituximab and adrenocorticotropic hormone analogues are the newer therapeutic options that should be considered in the therapy of primary MN.

  • membranous nephropathy
  • glomerulonephritis
  • cyclophosphamide
  • rituximab
  • nephrotic syndrome
  • Ponticelli

Statistics from Altmetric.com

Footnotes

  • Contributors KCK is the primary author. KCK, TC and VK performed literature review and drafted the manuscript. SB and LB edited and revised the final draft to completion.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.