Rituximab in pemphigus

Immunotherapy. 2018 Jan;10(1):27-37. doi: 10.2217/imt-2017-0104. Epub 2017 Oct 24.

Abstract

Pemphigus is a severe autoimmune blistering disease mediated by pathogenic anti-desmoglein antibodies leading to an inter keratinocyte disjunction. Rituximab is a monoclonal antibody that binds to the CD-20 antigen of B lymphocytes, which causes B-cell depletion and a subsequent reduction in pathogenic autoantibodies. Its therapeutic role in pemphigus has been progressively growing with increasing evidence of successful outcomes. Rituximab was initially off-labeled used as an alternative in patients with recalcitrant or relapsing pemphigus and in patients with contraindications to systemic corticosteroids. Recently, a large randomized clinical trial has shown that first-line use of rituximab combined with short-term prednisone regimen was both more effective and potentially safer than a standard regimen of high doses of corticosteroids in patients with moderate to severe pemphigus.

Keywords: B lymphocytes; CD20; anti-desmoglein antibodies; autoimmune regimen; corticosteroids; efficacy; pemphigus superficial; pemphigus vulgaris; rituximab; safety.

MeSH terms

  • Autoantibodies / immunology*
  • Autoantibodies / metabolism*
  • B-Lymphocytes / immunology*
  • Clinical Trials as Topic
  • Desmogleins / antagonists & inhibitors
  • Desmogleins / immunology
  • Drug Therapy, Combination
  • Humans
  • Intercellular Junctions / metabolism*
  • Intercellular Junctions / pathology
  • Keratinocytes / physiology*
  • Lymphocyte Depletion
  • Pemphigus / drug therapy*
  • Prednisone / therapeutic use
  • Rituximab / therapeutic use*

Substances

  • Autoantibodies
  • Desmogleins
  • Rituximab
  • Prednisone