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Predictors of long-term outcomes in pediatric focal segmental glomerulosclerosis
Yingchao Peng1, Chunlin Gao2, Chao Xu3
1Department of Pediatrics, Affiliated Jinling Hospital, Medical School of Nanjing University, Nanjing, China.
Insights
This study reveals that Chinese children with focal segmental glomerulosclerosis (FSGS) have a 10-year renal survival rate of 62.58%. The Columbia classification effectively predicts prognosis, with the collapsing variant and high tubulointerstitial damage indicating poorer outcomes.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Glomerular Diseases
Background:
- Limited data exists on primary focal segmental glomerulosclerosis (FSGS) in children, with short follow-up periods and small sample sizes.
- The application of the Columbia classification for pediatric FSGS requires further validation.
- This study addresses these gaps by analyzing a large cohort of Chinese children with primary FSGS.
Purpose of the Study:
- To determine the long-term prognosis and identify risk factors for primary focal segmental glomerulosclerosis (FSGS) in a substantial cohort of Chinese children.
- To evaluate the predictive value of the Columbia classification system in this pediatric population.
Main Methods:
- A cohort of 274 Chinese children with primary FSGS was studied retrospectively from 2003 to 2018.
- Kaplan-Meier analysis and Cox regression were used to assess renal survival and risk factors.
- Receiver operating characteristic (ROC) curve analysis validated the predictive accuracy of the Columbia classification and identified key prognostic indicators.
Main Results:
- The 10-year and 15-year renal survival rates were 62.58% and 34.66%, respectively.
- Significant predictors of poor renal outcome included chronic tubulointerstitial damage (≥25%), the collapsing variant of FSGS, and segmental sclerosis.
- Age at biopsy also correlated with renal outcomes, while the tip variant showed a better prognosis.
Conclusions:
- The Columbia classification is a valuable tool for predicting renal outcomes in Chinese children with FSGS.
- Chronic tubulointerstitial damage (≥25%) and the collapsing variant are associated with a poorer prognosis.
- The tip variant of FSGS is linked to a more favorable renal survival rate in this cohort.
Background:
Available data on primary focal segmental glomerulosclerosis (FSGS) in children usually report on short follow-up and small samples. Furthermore, the application of the Columbia classification for FSGS in children has not yet been fully agreed. We aimed to confirm the prognosis and risk factors of FSGS in a large cohort of Chinese children.
Methods:
Two hundred seventy-four children with primary FSGS from a single center were enrolled from 2003 to 2018. Long-term renal survival and related risk factors were evaluated by the Kaplan-Meier method and Cox multivariate regression analysis. Receiver operating characteristic (ROC) curve analysis further tested the effect of various risk factors in predicting renal outcomes. The composite end-point included ≥ 50% reduction in estimated glomerular filtration rate and/or end-stage renal disease or death.
Results:
One hundred twenty-five children were diagnosed with not otherwise specified (NOS) (45.6%) variant; 79 with tip lesions (28.8%), 32 with collapsing (11.7%), 31 with cellular (11.3%), and 7 with perihilar lesions (2.6%). The renal survival rate was 80.73% at 5 years, 62.58% at 10 years and 34.66% at 15 years. Multivariate analysis showed that chronic tubulointerstitial damage ≥ 25% (HR 4.14, 95% CI 1.49-11.50, P < 0.01), collapsing variant [(reference: NOS) HR 2.16, 95% CI 1.10-4.27, P = 0.03], segmental sclerosis (HR 1.03, 95% CI 1.01-1.04, P < 0.01) and age at biopsy (HR 0.91, 95% CI 0.85-0.98, P = 0.01) were significantly associated with renal outcomes. ROC curve analysis showed an excellent diagnostic yield of the Columbia classification. The combination of Columbia classification, CTI ≥ 25% and segmental sclerosis had the best predictive value for renal outcomes (AUC = 0.867, sensitivity = 77.78%, specificity = 82.27%, P < 0.01).
Conclusions:
This study reports a renal survival rate of Chinese children with FSGS of 62.58% at 10 years and 34.66% at 15 years. Prognosis is poorer in patients with collapsing variant or CTI ≥ 25% and good in patients with tip variant. The Columbia classification is confirmed as a valuable tool for predicting prognosis of Chinese children with FSGS.
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