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Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
Published on: June 8, 2022
Clinical outcomes of childhood lupus nephritis: a single center's experience
Byong Sop Lee1, Hee Yeon Cho, Eo Jin Kim
1Department of Pediatrics, Seoul National University Children's Hospital, 28 Yongon-dong, Chongno-gu, Seoul, 110-744, South Korea.
Insights
Pediatric lupus nephritis (LN) patients often respond to initial treatment, but flares and progression to chronic renal failure (CRF) remain significant concerns. Male gender, hypertension, and lack of remission are key predictors of CRF in class IV LN.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Lupus nephritis (LN) is a severe complication of childhood-onset systemic lupus erythematosus.
- Long-term outcomes and prognostic factors in pediatric LN require further elucidation.
Purpose of the Study:
- To retrospectively analyze the clinical course, treatment responses, and long-term outcomes of pediatric patients with lupus nephritis.
- To identify prognostic factors associated with the progression to chronic renal failure (CRF) in this population.
Main Methods:
- Retrospective review of medical records of 77 children diagnosed with lupus nephritis between 1986 and 2005.
- Analysis of initial biopsy findings (WHO classification), treatment regimens, clinical flares, and progression to CRF or end-stage renal disease (ESRD).
- Multivariate analysis and Kaplan-Meier survival estimates were used to identify risk factors for CRF and assess patient survival.
Main Results:
- Most patients (87.0%) initially responded to corticosteroids and/or immunosuppressants.
- 44.8% of responders experienced subsequent flares (proteinuric or nephritic).
- 16.9% progressed to CRF/ESRD, and 7.8% died. Male gender, initial hypertension, and absence of remission predicted CRF. Glomerulosclerosis (≥10%), nephritic flares, and antiphospholipid antibodies/syndrome were independent risk factors for CRF.
Conclusions:
- Pediatric lupus nephritis has a significant risk of flares and progression to chronic renal failure despite initial treatment responses.
- Male gender, hypertension, lack of remission, specific histological findings, and antiphospholipid syndrome are critical prognostic factors for CRF.
- Cyclophosphamide pulse therapy did not show superiority over other combined immunosuppressive regimens for diffuse proliferative LN.
Abstract:
This study retrospectively reviewed the medical records of children with lupus nephritis (LN) who were treated at Seoul National University Children's Hospital from 1986 to 2005 (mean duration 8.3+/-4.4 years). The records of 77 children (22 male and 55 female) were examined. The mean age at diagnosis was 11.9+/-3.0 years. The initial biopsy results revealed a WHO class IV classification for 60 (88.2%) of 68 biopsy proven cases. Of 77 patients, 67 (87.0%) responded initially to the high-dose corticosteroids with or without additional immunosuppressive therapy. Of the initial responders (67), 30 (44.8%) experienced at least one episode of proteinuric (24) or nephritic (6) flare. Thirteen patients (16.9%) progressed to either chronic renal failure (CRF) or end-stage renal disease (ESRD). Six (7.8%) patients died. A Kaplan-Meier estimate of patient survival and CRF-free survival rate was 95.4% and 88.7% at 5 years and 91.8% and 74.7% at 10 years, respectively. Multivariate analysis for class IV LN revealed male gender (P=0.029), initial hypertension (P=0.001) and absence of remission (P=0.002) to be prognostic factors predicting CRF. Glomerulosclerosis of 10% or more (P=0.005), nephritic flare (P=0.011), and presence of anti-phospholipid antibody (P=0.017) or syndrome (P=0.004) were also found to be independent risk factors for CRF. Cyclophosphamide pulse therapy failed to demonstrate superiority over other combined immunosuppressants used for the treatment of diffuse proliferative LN.
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