Targeting the B cell in vasculitis

Pediatr Nephrol. 2009 Jul;24(7):1267-75. doi: 10.1007/s00467-008-1010-z. Epub 2008 Oct 18.


The treatment of severe vasculitides, such as antineutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitis, is associated with both short- and long-term toxicities that limit its use in paediatric patients and is complicated by refractory and relapsing disease that requires increased exposure to these toxic therapies. B cells have been implicated in the pathogenesis of autoimmune diseases, including vasculitis, and represent a potential target for new therapies that may have a more acceptable toxicity profile. The use of rituximab, a biologic therapy directed against B cells, has provided valuable insight into the potential role of B-cell targeted therapies for vasculitis. Rituximab appears to be a potentially useful treatment for vasculitis in adult patients, but randomized evidence comparing it to cyclophosphamide in terms of both efficacy and toxicity is lacking, as is long-term safety data. Several other B-cell-directed therapies are in development and may offer rational alternatives or adjunctives to traditional treatments.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Antibodies, Antineutrophil Cytoplasmic / immunology
  • Antibodies, Monoclonal / therapeutic use*
  • Antibodies, Monoclonal, Murine-Derived
  • Autoantigens / immunology
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology
  • B-Lymphocytes / drug effects
  • B-Lymphocytes / immunology*
  • Child
  • Clinical Trials as Topic
  • Humans
  • Immunologic Factors / therapeutic use*
  • Rituximab
  • Vasculitis / drug therapy*
  • Vasculitis / immunology*


  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Autoantigens
  • Immunologic Factors
  • Rituximab