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Abstract: TH-PO1016

Immunosuppressive Therapy for Children with Membranous Nephropathy: A Report from the Cure Glomerulopathy Network (CureGN)

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Laurin, Louis-Philippe, Hopital Maisonneuve-Rosemont, Montreal, Quebec, Canada
  • Ashoor, Isa, Children's Hospital of New Orleans, New Orleans, Louisiana, United States
  • Srivastava, Tarak, Childrens's Mercy Hospital, Kansas City, Missouri, United States
  • Al-Uzri, Amira, Oregon Health & Science University, Portland, Oregon, United States
  • Troost, Jonathan P., University of Michigan, Ann Arbor, Michigan, United States
  • Rheault, Michelle N., University of Minnesota, Minneapolis, Minnesota, United States
  • Bou Matar, Raed, Cleveland Clinic, Cleveland, Ohio, United States
  • Gibson, Keisha L., University of North Carolina Kidney Center, Chapel Hill, North Carolina, United States
  • Selewski, David T., University of Michigan, Ann Arbor, Michigan, United States
  • Gipson, Debbie S., University of Michigan Mott Children's Hospital, Ann Arbor, Michigan, United States
  • Wang, Chia- Shi, Emory University School of Medicine, Atlanta, Georgia, United States

Group or Team Name

  • CureGN Membranous Epi Treated vs. Not Treated Work Group
Background

Membranous nephropathy (MN) is a rare cause of nephrotic syndrome in children. Current practice patterns regarding choice and timing of immunosuppressive therapy (IST) remain largely undescribed.

Methods

CureGN is an ongoing multi-center prospective, observational cohort of children and adults with biopsy-proven MCD, FSGS, MN, or IgAN/IgAV. Biopsies were performed between 2010 and 2017. Descriptive statistics were used to assess choice and timing of IST after incident kidney biopsy. Categorical data are presented as frequencies and percentages and compared using chi-square tests; continuous data as median(IQR) and compared using the Kruskal-Wallis test.

Results

Forty-one children with MN were enrolled. Thirty participants without pre-biopsy IST exposure (N=29) or exposed to steroids only within 6 weeks before biopsy (N=1) were treated as IST naïve and included in the analysis. Median age at time of biopsy was 14 yrs (12-16). 57% were female. Median urine protein to creatinine ratio (UP:C) was 3.8 (1.3-7.4), serum albumin was 2.2 g/dl (1.7-3.1), and estimated GFR was 107 mL/min/1.73m2 (84-137). 21/30 (70%) of patient received IST (Table 1), with 81% of IST treatments started in the first 6 months after biopsy. 15/30 (50%) were treated with RAAS blockers by 6 months. Treated patients with IST (vs. untreated) by 6 months after biopsy had similar UP:C (3.8 vs. 3.4 g/g; p=0.60), serum albumin (2.4 vs. 2.1 g/dl; p=0.81) and eGFR (119 vs. 100; p=0.56) at time of biopsy.

Conclusion

Corticosteroids and calcineurin inhibitors were the main agents used in children with biopsy-proven MN. Therapies were started early within the first 6 months after biopsy in 81% of patients, deviating from current KDIGO practice guidelines.

Table 1. First IST used in pediatric-onset MN patients with no IST treatment before biopsy (n=30)

Funding

  • NIDDK Support