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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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Long-term renal outcomes of childhood-onset global and segmental diffuse proliferative lupus nephritis

Pornpimol Rianthavorn1, Athitaya Buddhasri2

  • 1Division of Nephrology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, 1873 King Rama 4 Road, Pathumwan, Bangkok, 10330, Thailand. Pornpimol.R@chula.ac.th.

Pediatric Nephrology (Berlin, Germany)
|June 10, 2015
PubMed

Insights

This study found that diffuse proliferative lupus nephritis (DPLN) in children, whether global (IV-G) or segmental (IV-S), shows similar long-term renal survival despite some clinical differences. More research is needed to slow chronic kidney disease progression in these pediatric patients.

Area of Science:

  • Pediatric Nephrology
  • Rheumatology
  • Immunology

Background:

  • Limited data exists on global (IV-G) and segmental (IV-S) diffuse proliferative lupus nephritis (DPLN) in pediatric populations.
  • Understanding the clinicopathology and prognosis of these DPLN subclasses is crucial for effective management.

Purpose of the Study:

  • To investigate the clinicopathological features and long-term prognosis of DPLN subclasses IV-G and IV-S in children.
  • To compare the outcomes of pediatric patients with DPLN IV-G versus IV-S.

Main Methods:

  • Retrospective analysis of clinical, laboratory, and demographic data from 56 children (<18 years) diagnosed with DPLN between 2004 and 2013.
  • Categorization into IV-G (36 patients) and IV-S (20 patients) subclasses.
  • Evaluation of clinical endpoints including complete remission (CR), chronic kidney disease (CKD), and death.

Main Results:

  • Patients with IV-G showed higher proteinuria and activity index compared to IV-S.
  • Global endocapillary proliferation was predominant in IV-G, while segmental endocapillary proliferation was more common in IV-S.
  • Complete remission rates were 50% for IV-G and 60% for IV-S. Renal and patient survival rates were similar between subclasses at 1, 5, and 10 years.

Conclusions:

  • Despite clinical and histopathological differences, DPLN subclasses IV-G and IV-S exhibit similar renal outcomes in children.
  • Most children with DPLN survive to adulthood but experience significant renal damage.
  • Development of treatment regimens to slow CKD progression in pediatric DPLN is essential.
Abstract

Keywords:
ChildrenChronic kidney diseaseComplete remissionEnd-stage renal diseaseSurvival

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