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Clinicopathologic features of non-lupus membranous nephropathy in a pediatric population

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Abstract

Background

Membranous nephropathy is an uncommon cause of nephrotic syndrome in pediatrics.

Methods

We reviewed our kidney biopsy records for patients ≤ 20 years of age with membranous nephropathy without evidence of systemic lupus erythematosus within 6 months of biopsy (January 1995–September 2020). Staining for PLA2R, NELL1, THSD7A, SEMA3B, EXT2 (3 biopsies), and IgG-subclass were performed.

Results

Sixteen children (≤ 12 years) and 25 adolescents (13–20 years) were identified. Four children and 15 adolescents showed autoantigen positivity: PLA2R+/SEMA3B- (13), SEMA3B+/PLA2R+ (2), SEMA3B+/PLA2R− (1), NELL1 (1), EXT2+ (2), and THSD7A (0). Co-morbidities associated with PLA2R positivity included IPEX syndrome, active hepatitis B, Von Hippel Lindau syndrome, solitary kidney, type 1 diabetes, hyperuricemia, pregnancy (1), obesity (3), type II diabetes, H. pylori, viral prodrome, and nephrolithiasis. The SEMA3B+/PLA2R− adolescent was pregnant, the NELL1+ adolescent was obese, and the two EXT2+ adolescents eventually met the clinical criteria for lupus (4, 9 years post-biopsy). Co-morbidities among the remaining 24 patients included remote hepatitis B (2), Down’s syndrome, lysinuric protein intolerance, recurrent UTIs, hypothyroidism, pregnancy (3), and obesity (2). Follow-up data was available for 12 children and 16 adolescents. Of the 12 children, 6 achieved complete remission, 4 achieved partial remission, and 2 had no response to treatment (1 transplant). Of the 16 adolescents, 4 achieved complete remission, 4 achieved partial remission, and 8 had no response to treatment (3 transplants). A child with “full-house” immunofluorescence staining achieved spontaneous disease remission.

Conclusion

Our non-lupus membranous nephropathy cohort represents one of the largest pediatric studies to date.

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Author notes

  1. Paul Miller and Li Lei contributed equally.

Authors and Affiliations

  1. Department of Pathology, Stanford University, H2110, 300 Pasteur Drive, Stanford, CA, 94305, USA

    Paul Miller, Li Lei, Vivek Charu, John Higgins, Megan Troxell & Neeraja Kambham

  2. UC Davis, Sacramento, CA, USA

    Li Lei

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Miller, P., Lei, L., Charu, V. et al. Clinicopathologic features of non-lupus membranous nephropathy in a pediatric population. Pediatr Nephrol 37, 3127–3137 (2022). https://doi.org/10.1007/s00467-022-05503-7

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