Principles of pediatric lupus nephritis in a prospective contemporary multi-center cohort

Kathleen M Vazzana1, Ankana Daga2, Beatrice Goilav3

  • 1Lupus Genomics and Global Disparities Unit, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.

Lupus
|July 5, 2021
PubMed

Insights

Lupus nephritis (LN) affects 32% of children with childhood-onset systemic lupus erythematosus (cSLE). Black children experienced worse kidney outcomes, though most patients saw stable or improved kidney function short-term.

Area of Science:

  • Pediatric Rheumatology
  • Nephrology
  • Immunology

Background:

  • Lupus nephritis (LN) is a severe complication of childhood-onset systemic lupus erythematosus (cSLE).
  • Changing epidemiology and management of cSLE necessitate updated understanding of LN outcomes.
  • Historical principles of LN require validation in current pediatric cohorts.

Purpose of the Study:

  • To validate established LN principles in a contemporary cSLE cohort using the CARRA registry.
  • To assess LN incidence, timing, short-term renal outcomes, and treatment patterns in cSLE.
  • To identify risk factors and prognostic indicators for LN in children.

Main Methods:

  • Utilized the Childhood Arthritis and Rheumatology Research Alliance (CARRA) prospective registry.
  • Evaluated 677 cSLE patients for documented LN and associated factors.
  • Analyzed LN incidence, time to diagnosis, short-term renal function, and rituximab use.

Main Results:

  • 32% of cSLE patients in the registry had documented LN.
  • Black cSLE patients showed a higher incidence of kidney function decline and worse short-term renal outcomes.
  • Most children with LN had stable or improved kidney function in the short term; end-stage kidney disease (ESKD) was rare.

Conclusions:

  • Findings validate some historical LN principles in cSLE but highlight racial disparities in outcomes.
  • Short-term renal outcomes for pediatric LN appear favorable, with rare ESKD.
  • Long-term follow-up of the CARRA cohort is crucial for understanding the full prognosis of LN in cSLE.

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