Modern therapeutic strategies for paediatric systemic lupus erythematosus and lupus nephritis

Acta Paediatr. 2010 Jul;99(7):967-74. doi: 10.1111/j.1651-2227.2010.01771.x. Epub 2010 Mar 11.

Abstract

There is still a significant morbidity and mortality associated with childhood-onset systemic lupus erythematosus (SLE), despite an increasing armamentarium of immunosuppressive agents. The ideal therapeutic strategy for children and adolescents with SLE should provide the right amount of treatment to allow normal growth, development and fertility while reducing the disease activity and damage that can be accrued over the years. Each patient should have individualized treatments tailored to their organ involvement, disease severity and history of flares together with recent clinical, haematological and immunological parameters to avoid further flares of disease activity and side-effects of treatment, especially severe infections and future malignancies. The most commonly cited side-effects of medications include Cushingoid features of corticosteroids, infective complications of cyclophosphamide and gastrointestinal side-effects of mycophenolate mofetil. There is increasing evidence to support the use of oral mycophenolate mofetil as opposed to cyclophosphamide for both induction and maintenance therapies in many children with SLE with or without lupus nephritis (LN). Recently, case series utilizing B-lymphocyte depletion therapies with rituximab look promising for patients with severe or refractory disease activity. In this article, we explore current evidence to effectively treat children and adolescents with SLE with or without LN.

Conclusion: Modern therapeutic strategies include reduced doses and use of corticosteroids and intravenous cyclophosphamide respectively, with increased use of azathioprine, MMF and rituximab.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Murine-Derived
  • B-Lymphocytes / drug effects
  • Child
  • Cyclophosphamide / therapeutic use
  • Humans
  • Immunologic Factors / therapeutic use
  • Immunosuppressive Agents / therapeutic use*
  • Lupus Erythematosus, Systemic / therapy*
  • Lupus Nephritis / therapy*
  • Plasma Exchange
  • Remission Induction
  • Rituximab

Substances

  • Adrenal Cortex Hormones
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Immunologic Factors
  • Immunosuppressive Agents
  • Rituximab
  • Cyclophosphamide