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Lupus nephritis in children: a longitudinal study of prognostic factors and therapy
1Department of Pediatrics, State University of New York Health Science Center at Brooklyn, NY 11203, USA.
Insights
Childhood lupus nephritis carries a high risk of kidney failure. Class IV histology, hypertension, and low C3 complement levels at diagnosis are key indicators of progression to end-stage renal disease (ESRD).
Area of Science:
- Pediatric Nephrology
- Autoimmune Diseases
- Renal Pathology
Background:
- Childhood lupus nephritis is a serious condition with limited research on risk factors for renal failure.
- Understanding prognostic indicators is crucial for managing pediatric patients with lupus nephritis.
Purpose of the Study:
- To identify risk factors for renal failure in children diagnosed with lupus nephritis.
- To analyze the relationship between initial clinical and histological factors and disease progression.
- To evaluate the impact of different treatment regimens on patient outcomes.
Main Methods:
- Retrospective review of 56 children (4-18 years) with lupus nephritis over a 27-year period (1965-1992).
- Analysis of percutaneous renal biopsy histology, clinical data (serum creatinine, proteinuria, hypertension), and serological markers (C3 complement, DNA antibodies).
- Life-table analysis and univariate/multivariate analyses to determine associations with end-stage renal disease (ESRD).
Main Results:
- Cumulative survival was 82.8% at 5 years and 67.7% at 10 years; renal survival was 44.4% at 5 years and 29% at 10 years.
- Univariate analysis linked elevated creatinine, decreased C3 complement, hypertension, and Class IV histology to ESRD progression.
- Multivariate analysis identified initial Class IV histology (RR 1.78), hypertension (RR 1.67), and low C3/high creatinine (RR 1.52) as independent predictors of ESRD.
Conclusions:
- Children with lupus nephritis, particularly those with Class IV disease, hypertension, high creatinine, and low C3 levels at diagnosis, face a higher risk of ESRD.
- Initial histological classification is the most reliable prognostic factor for disease progression in pediatric lupus nephritis.
- The study did not find a significant difference in outcomes between the two treatment groups examined.
Abstract:
There are only a few studies in the pediatric literature that have analyzed risk factors for renal failure in childhood lupus nephritis. This study reviewed the outcome of 56 children (4 to 18 yr of age) with lupus nephritis seen at the authors' institution over a 27-yr period (1965 to 1992), in relation to risk factors and therapy. All children underwent percutaneous renal biopsy before the institution of therapy. From 1965 to 1987, treatment for Class III and IV lupus nephritis consisted of high-dose pulse methylprednisolone, 500 mg daily for 10 days, followed by oral prednisone. From 1987 to 1992, IV cyclophosphamide was given monthly for 6 months and then every 3 months for a period of 3 yr for patients with Class III and Class IV disease. Of 56 children, 42% had Class IV and 21% had Class III histology at onset. The mean follow-up period was 4 yr and ranged from 0.5 to 20.3 yr. Life-table analysis showed that the cumulative proportion of patients surviving was 82.8% at 5 yr and 67.7% at 10 yr. Renal survival was 44.4% at 5 yr and 29% at 10 yr, after the initial diagnosis of lupus nephritis was made. Age at diagnosis, race, sex, initial serum creatinine level, and the presence of proteinuria, hypertension, and DNA antibody titers were reviewed with respect to disease progression, as was the histological class at diagnosis. The effect of the different therapies was also examined. Univariate analysis revealed a significant association of progression to ESRD with an elevated serum creatinine level (P = 0.021), decreased C3 complement (P = 0.024), hypertension (P = 0.053), and histological classification of Class IV lupus nephritis (P = 0.031). Multivariate analysis demonstrated that progression to ESRD was independently associated with an initial Class IV histology (relative risk, 1.78; P < 0.003), hypertension at presentation (relative risk, 1.67; P < 0.003), and a low C3 complement level in conjuction with a high creatinine level (relative risk, 1.52; P < 0.028). Among children with lupus nephritis, those with Class IV disease, hypertension, high creatinine levels, and low C3 complement levels at the time of diagnosis are at increased risk for ESRD. Initial histological classification of lupus nephritis was the most reliable prognostic factor for disease progression. This study was unable to detect a difference in outcome for the two treatment groups.