Emerging therapeutic strategies for minimal change disease and focal and segmental glomerulosclerosis

Expert Opin Investig Drugs. 2018 Nov;27(11):839-879. doi: 10.1080/13543784.2018.1540587. Epub 2018 Oct 30.

Abstract

Introduction: Minimal change disease (MCD) and Focal and segmental glomerulosclerosis (FSGS) are two of the major causes of nephrotic syndrome (NS) in children and adults. According to KDIGO (Kidney Disease: Improving Global Outcomes) guidelines, the treatment of adult primary MCD and FSGS should be based on immunosuppressants and antiproteinuric drugs. Recently, Rituximab, a humanized monoclonal antibody (mAb) has emerged as a potential treatment for steroid or calcineurin inhibitor-dependent patients; it has however demonstrated lower efficacy in those with nephrotic syndrome that is resistant to the above indicated drugs.

Areas covered: Analysis of ongoing and already completed clinical trials, retrieved from clinicaltrials.gov, clinicaltrialsregister.eu and PubMed involving new therapies for nephrotic syndrome secondary to MCD and FSGS.

Expert opinion: The most promising drugs under investigation for MCD and FSGS are mAbs. We are hopeful that new therapeutic options to treat multi-drug resistant MCD and FSGS will emerge from currently ongoing studies. What appears certain is the difficulty in enrolling patients affected by orphan renal diseases and the selection of valid endpoints in clinical trials, such as kidney failure.

Keywords: FSGS; Focal and segmental glomerulosclerosis; MCD; minimal change disease; nephrotic syndrome; novel therapies.

Publication types

  • Review

MeSH terms

  • Adult
  • Animals
  • Antibodies, Monoclonal / therapeutic use
  • Child
  • Drug Design
  • Drug Resistance, Multiple
  • Glomerulosclerosis, Focal Segmental / complications
  • Glomerulosclerosis, Focal Segmental / drug therapy*
  • Humans
  • Immunologic Factors / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Nephrosis, Lipoid / complications
  • Nephrosis, Lipoid / drug therapy*
  • Nephrosis, Lipoid / therapy
  • Nephrotic Syndrome / etiology
  • Nephrotic Syndrome / prevention & control*
  • Practice Guidelines as Topic
  • Proteinuria / drug therapy
  • Proteinuria / physiopathology
  • Rituximab / therapeutic use

Substances

  • Antibodies, Monoclonal
  • Immunologic Factors
  • Immunosuppressive Agents
  • Rituximab