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Updated: Oct 30, 2025

Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
Published on: June 8, 2022
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.
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.
Abstract:
Lupus nephritis (LN) is a life-threatening manifestation of systemic lupus erythematosus (SLE) and is more common in children than adults. The epidemiology and management of childhood-onset SLE (cSLE) have changed over time, prompting the need to reassess expected outcomes. The purpose of this study is to use the Childhood Arthritis and Rheumatology Research Alliance (CARRA) prospective registry to validate historical principles of LN in a contemporary, real-world cohort. After an extensive literature review, six principles of LN in cSLE were identified. The CARRA registry was queried to evaluate these principles in determining the rate of LN in cSLE, median time from cSLE diagnosis to LN, short-term renal outcomes, and frequency of rituximab as an induction therapy. Of the 677 cSLE patients in the CARRA registry, 32% had documented LN. Decline in kidney function was more common in Black cSLE patients than non-Black patients (p = 0.04). Black race was associated with worse short-term renal outcomes. In short-term follow up, most children with LN had unchanged or improved kidney function, and end stage kidney disease (ESKD) was rare. Ongoing follow-up of cSLE patients in the CARRA registry will be necessary to evaluate long-term outcomes to inform risk, management, and prognosis of LN in cSLE.
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