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Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
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Membranous nephropathy (MN) accounts for <5% of pediatric nephrotic syndrome. PLA2R-positive MN represents 70-80% of primary cases, but tissue-serum discordance occurs in 20-30% and may predict treatment resistance. While rituximab achieves 60% remission, 30-40% show suboptimal response. Modified Ponticelli regimen achieves 60-85% remission with durable responses. This case demonstrates rituximab resistance, CNI intolerance, and successful Ponticelli salvage in an adolescent with tissue-serum discordance and latent TB.
A 16-year-old female (52kg) presented with severe nephrotic syndrome (PCR 8g/g, albumin 1.3g/dL). Biopsy confirmed PLA2R-positive Stage II MN with persistently negative serum antibodies (tissue+/serum-). Following household TB exposure she was screened and found to be positive for interferon gamma release assay (IGRA), she completed 3HP (once weekly Isoniazid -Rifapentine for 3 months) prophylaxis before rituximab (800mg=15.4mg/kg). Despite confirmed B-cell depletion (<1% CD19+) at 3 and 8 months, remission was not achieved. Tacrolimus failed due to GI intolerance. Modified Ponticelli initiated: alternating monthly cycles of (1) IV methylprednisolone 250mg×3 days then oral prednisolone 0.5mg/kg/day×27 days (months 1,3,5), and (2) oral cyclophosphamide 1.5mg/kg/day×30 days (months 2,4,6). Cumulative cyclophosphamide 6750mg (135mg/kg), below gonadotoxicity threshold was maintained.
At 1-month post-Ponticelli, patient achieved early partial remission: PCR improved 87.5% (8→1g/g), albumin significantly improved (1.3→3.0g/dL), complete edema resolution, lipid normalization (cholesterol 280→198mg/dL, LDL 230→128mg/dL), stable eGFR (145→151mL/min/1.73m²). Treatment well-tolerated: nadir WBC 3,200/μL, ANC 1,800/μL, platelets 142,000/μL, peak ALT 62U/L, hemoglobin 11g/dL, no infections or hemorrhagic cystitis. Functional capacity improved from inability to attend school to regular daily attendance. .
Modified Ponticelli achieved early partial remission (87.5% proteinuria reduction) in rituximab-resistant, CNI-intolerant adolescent PLA2R-positive membranous nephropathy following TB prophylaxis. Tissue-serum PLA2R discordance might predict rituximab resistance despite confirmed B-cell depletion with low-dose protocol (15.4mg/kg) based on Asian cohort studies, suggesting antibody consumption or immune complex masking mechanisms. Severe hypoalbuminemia complicated CNI absorption. Safety profile was acceptable with transient cytopenias, no infections, and cumulative cyclophosphamide below gonadotoxicity threshold. IGRA screening and TB prophylaxis completion were mandatory before immunosuppression in this TB-endemic setting, critical risk-benefit assessment balancing infection risk against severe disease burden was essential here . Complete remission is anticipated within 6-12 months based on typical MN response . Restoration of daily school attendance demonstrates meaningful quality-of-life impact. This case underscores conventional immunosuppression relevance when biologics fail and the importance of individualized treatment strategies which integrates efficacy, safety, accessibility, cost-effectiveness, and infectious disease screening in adolescent glomerular disease management.
