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Kidney Week

Abstract: FR-PO0808

Membranous Nephropathy in Pediatric Patients: Characterization and Long-Term Outcomes of the CureGN Cohort

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Giang, Sophia, Stanford University, Stanford, California, United States
  • Helmuth, Margaret, University of Michigan, Ann Arbor, Michigan, United States
  • Khalid, Myda, University of Iowa Health Care, Iowa City, Iowa, United States
Background

Pediatric primary membranous nephropathy (ppMN) is a rare diagnosis, comprising only 3-7% of childhood glomerulonephritis. Its clinical course ranges from spontaneous remission to ESKD with little understanding of the risk factors for these outcomes. Our aims are to 1) characterize features and trajectories of the largest US ppMN cohort, and 2) identify predictors of long-term outcomes.

Methods

We utilized data from CureGN, a prospective, longitudinal study of children and adults with glomerular disease, between 2014 and 2026. We explored the relationship between age of disease onset (young children <12 vs adolescent >=12) and various clinical factors and used Kaplan Meier curves to visualize progression to >=30% eGFR decline and disease remission (proteinuria <0.3g/g). Independent predictors of >=30% eGFR decline and remission were identified using univariate Cox regression.

Results

The cohort included 61 children diagnosed at a median age of 14 years with median follow up of 5.1 years (IQR 2.6-7.7). The median time between diagnosis and enrollment was 0.4 (0.2,1.6) years. 49.2% were female; 63.9% were White with the largest minority being Black (18%); 26.2% were Hispanic. Most children had BMI >25 at the time of enrollment (59.3%). Adolescents were more likely have hypertension (46.7% vs 18.8% p =0.049) and PLA2R positivity (51% vs 27%, p =0.16). 20% and 13.1% of subjects had >=30% eGFR decline and ESKD, respectively, with similar proportions by age group. Of 41 patients who had proteinuria >3g/g during follow up, 59% achieved remission over a median of 2.6 (1.1,6.1) years (Figure 1). Independent risk factors for outcomes are summarized in Table 1.

Conclusion

20% of children had >=30% eGFR loss/ESKD during follow-up. Higher UPCR at enrollment and PLA2R positivity were associated with poorer outcomes.

Table 1
 Remission>=30% eGFR decline
Predictors*HR (95% CI)P-valueHR (95% CI)P-value
<12 years at biopsy1.68 (0.69,4.10)0.250.73 (0.24,2.22)0.57
UPCR** (per UPCR doubling)0.94 (0.81,1.09)0.411.65 (1.17,2.35)0.005
eGFR** (per 10ml/min/1.73m2)1.08 (0.96,1.21)0.210.88 (0.73,1.06)0.17
Black race0.59 (0.22,1.60)0.301.58 (0.47,5.36)0.46
PLA2R positivity0.62(0.23,1.48)0.285.44 (1.45,20.40)0.012

*Each predictor is modeled independently of others **At time of enrollment