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Abstract: FR-PO855

Pediatric Patients with Membranous Glomerulopathy: A Single-Center Retrospective Study

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Kouri, Anne, Indiana University School of Medicine, Indianapolis, Indiana, United States
  • Phillips, Carrie L., Indiana University School of Medicine, Indianapolis, Indiana, United States
  • Eadon, Michael T., Indiana University Division of Nephrology, Indianapolis, Indiana, United States
  • Andreoli, Sharon P., Indiana University, Indianapolis, Indiana, United States
  • Khalid, Myda, Indiana University, Indianapolis, Indiana, United States
Background

Membranous nephropathy is a rare cause of pediatric nephrotic syndrome. The purpose of this study is to characterize the clinical presentation, treatment and outcomes of pediatric patients with membranous glomerulopathy in a single center over a 15 year period.

Methods

Patients with membranous nephropathy, age 18 years or less, were identified through an existing database of renal biopsy specimens at Indiana University and through billing records. Their charts were reviewed for clinical presentation, treatment and outcomes data.

Results

From 2002 to 2019, there were a total of 777 renal biopsy specimens interpreted at Indiana University with a diagnosis of membranous nephropathy. Of these, there were 14 patients, age 18 years or less, identified with primary membranous nephropathy and met inclusion criteria. An additional 4 patients were identified from billing data.

Our cohort was primarily female (12/18) with a median age at presentation of 13 years (IQR 10.5--15.5). At presentation, nephrotic range proteinuria was present in 72%, hypoalbuminemia in 70%, edema in 62%, hematuria in 29%, and hypertension in 43% of patients. Renal function was normal in 17/18 patients at presentation. 3 patients had PLA2-R staining on biopsy and 2 were positive.

15 patients had treatment and/or outcomes data available. Median time to most recent follow-up was 20 months (IQR 7—35). 10/15 patients were treated with steroids; 12/15 had documented partial or complete remission at some point during their clinical course. Of the available data, 80%, 36%, and 15% had nephrotic range proteinuria at 3, 6, and 12 months, respectively. 27% and 38.5% of patients had entered complete remission at 6 and 12 months, respectively. One patient was steroid-dependent and relapsed when steroids were stopped. 14/15 patients were treated with an ACEi and/or ARB. One patient was treated with a steroid-sparing agent, mycophenolic acid, and did not enter remission. 14/15 patients had a creatinine less than 1.0 mg/dL at last follow-up. There were no documented complications of nephrotic syndrome such has thrombosis.

Conclusion

In our cohort, pediatric patients with membranous nephropathy presented with similar clinical characteristics as adult patients. Our patients had a good clinical response to steroids and/or ACEi/ARB with few documented adverse effects.