Article Text

other Versions

PDF
New insights into the pathogenesis and management of lupus in children
  1. A Midgley1,
  2. L Watson1,2,
  3. M W Beresford1,3
  1. 1Department of Women's and Children's Health, Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK
  2. 2Department of Paediatric Nephrology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK
  3. 3Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK
  1. Correspondence to Professor M W Beresford, Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UK; m.w.beresford{at}liverpool.ac.uk

Abstract

Systemic lupus erythematosus (SLE) is the archetypal systemic autoimmune disease, characterised by inflammation causing a wide spectrum of major clinical manifestations that may affect any organ. Childhood-onset SLE (cSLE) is more severe with greater damage and drug burden than adult-onset SLE. Understanding the pathogenesis of cSLE is a key step in directing medical management. The dysregulated immune system, that in health is usually vital in protecting the body from infection, contributes significantly to the disease process. Improved knowledge of disease mechanism will help to identify potential targets for novel agents and the identification of new biomarkers of disease activity. This review will present current knowledge of the innate and adaptive immune responses in cSLE and the optimal patient management that aims to control the disease. Innate immune dysregulation includes the overexpression of interferon-α, dendritic cell activation, neutrophil extracellular traps and phagocyte abnormalities. The classical adaptive immune system is over activated in lupus with excessive autoantibody production due to abnormalities in B and T cell regulation. Novel biologic medications are being developed to specifically target these areas with the ultimate aim of improving the long-term outlook and quality of life for children living with Lupus.

Statistics from Altmetric.com

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.