[Membranous glomerulonephritis (MGN), ongoing studies]

G Ital Nefrol. 2017 Sep 28;34(5):113-118.
[Article in Italian]

Abstract

The membranous nephropathy (MN) is the major cause of nephrotic syndrome in in the adult, account for 20% of cases with annual incidence is 1 in 100.000. In the past 10 years, the role of podocytes has been identified; environmental triggers in genetically predisposed patients can activate podocytes to exhibit antigenic epitopes (receptor of phospholipase A2, thrombospondin type 1) that become targets of specific autoantibodies with subsequent complement activation. The discovery of this mechanisms has opened new horizons in the therapy of MN and novel drugs are available with more specific mechanism of action. Rituximab, a monoclonal antibody directed against CD20 expressed by lymphocytes B, has been used in several trials and appears able to induce remission of nephrotic syndrome in 60% of patients (GEMRITUX trial) with similar risk profile. Nowadays it remains to define the most effective therapeutic pattern. In MN, the concept of targeting disease control, has permit novel therapies with specific blocking mechanisms (belimumab) and non-specific (ACTH) and new therapeutic options, such as ofatumumab, bortezomib and eculizumab, that have allowed to recognize pathological processes involved in the glomerular diseases.

Keywords: ECULIZUMAB; Membranous Glomerulonephritis; Rituximab; membranous nephropathy.

Publication types

  • Review

MeSH terms

  • Adrenocorticotropic Hormone / therapeutic use
  • Adult
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Autoantibodies / immunology
  • Autoantigens / immunology
  • Bortezomib / therapeutic use
  • Calcineurin Inhibitors / therapeutic use
  • Clinical Trials as Topic
  • Complement Activation
  • Cyclophosphamide / therapeutic use
  • Glomerulonephritis, Membranous / complications
  • Glomerulonephritis, Membranous / drug therapy*
  • Glomerulonephritis, Membranous / epidemiology
  • Glomerulonephritis, Membranous / immunology
  • Humans
  • Immunosorbent Techniques
  • Multicenter Studies as Topic
  • Nephrotic Syndrome / etiology
  • Observational Studies as Topic
  • Podocytes / immunology
  • Randomized Controlled Trials as Topic
  • Receptors, Phospholipase A2 / immunology
  • Rituximab / therapeutic use

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • Autoantibodies
  • Autoantigens
  • Calcineurin Inhibitors
  • Receptors, Phospholipase A2
  • Rituximab
  • Bortezomib
  • belimumab
  • Cyclophosphamide
  • Adrenocorticotropic Hormone
  • eculizumab
  • ofatumumab