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Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
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Three decades of progress in treating childhood-onset lupus nephritis

Tanya Pereira1, Carolyn L Abitbol, Wacharee Seeherunvong

  • 1Division of Pediatric Nephrology, University of Miami/Holtz Children's Hospital, Miami, FL 33101, USA.

Clinical Journal of the American Society of Nephrology : CJASN
|July 30, 2011
PubMed

Insights

Childhood lupus nephritis outcomes have improved with modern treatments. Mycophenolate mofetil significantly boosted renal and patient survival, highlighting advances in pediatric lupus nephritis care.

Area of Science:

  • Pediatric Nephrology
  • Autoimmune Diseases
  • Immunology

Background:

  • Childhood-onset lupus nephritis (LN) presents a poorer renal prognosis than adult-onset disease.
  • Controlled treatment trials in pediatric LN are notably lacking.
  • This study analyzes renal and patient survival over three decades in a pediatric LN cohort.

Purpose of the Study:

  • To compare renal and patient survival in childhood-onset lupus nephritis.
  • To evaluate the impact of evolving immunosuppressive treatment regimens on outcomes.
  • To identify prognostic factors for disease progression in pediatric LN.

Main Methods:

  • Retrospective analysis of 138 pediatric patients with systemic lupus erythematosus (1980-2010).
  • Core cohort of 95 patients with severe LN stratified by progression to end-stage renal disease (ESRD).
  • Patients categorized into four treatment eras based on primary immunosuppressive agents: corticosteroids (CS), cyclophosphamide (CYC), azathioprine (AZA), mycophenolate mofetil (MMF), and rituximab (RTX).

Main Results:

  • Poor renal function and nephrotic proteinuria at diagnosis predicted a worse prognosis.
  • Increasing proteinuria correlated with kidney disease progression.
  • The introduction of mycophenolate mofetil (MMF) in era 3 improved 5-year renal survival from 52% to 91% and overall patient survival from 83% to 97%.

Conclusions:

  • Renal and patient survival in childhood-onset lupus nephritis have significantly improved over the past three decades.
  • Progressive treatment regimens, including MMF, have contributed to better outcomes.
  • Further controlled clinical trials in pediatric LN are essential to optimize management.
Abstract

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