CASE REPORT article
Front. Med.
Sec. Nephrology
Pegcetacoplan-induced Remission in Pediatric Immune-Complex Membranoproliferative Glomerulonephritis with Comorbid Autosomal Recessive Polycystic Kidney Disease: A Case Report
Reem Alrasheed
Abdulkarim Alanazi
Raghad Bukhari
Sawsan Albatati
Hassan Faqeehi
Saeed Alzabali
King Fahd Medical City, Riyadh, Saudi Arabia
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Abstract
Background: Membranoproliferative glomerulonephritis (MPGN) is a rare glomerular disorder characterized by immune complex or complement-mediated injury, often leading to nephrotic syndrome, hypertension, and progressive renal dysfunction. Its management remains challenging, particularly in pediatric patients with coexisting renal pathologies. Case Presentation: We report a case of an 11-year-old girl who presented with nephrotic syndrome, severe hypertension, and impaired renal function. Renal biopsy confirmed immune complex-mediated MPGN (IC-MPGN), and genetic testing revealed a homozygous, pathogenic missense PKHD1 variant (NM_138694.3:c.4870C>T; p.(Arg1624Trp), leading to a diagnosis of autosomal recessive polycystic kidney disease (ARPKD). Initial treatment with prednisolone and mycophenolate mofetil failed to halt disease progression, and the patient became dialysis dependent. Pegcetacoplan, a complement C3 inhibitor, was subsequently initiated. After 11 weeks of pegcetacoplan therapy, the patient achieved renal recovery and was successfully weaned from dialysis. Proteinuria decreased from nephrotic to sub-nephrotic levels without significant adverse effects. Conclusion: To our knowledge, this is the first pediatric case of IC-MPGN with genetically confirmed ARPKD successfully treated with pegcetacoplan. The case illustrates that renal recovery occurred following initiation of proximal complement inhibition with pegcetacoplan.
Summary
Keywords
Autosomal recessive polycystic kidneydisease, Membranoproliferative glomerulonephritis, Pediatrics, Pegcetacoplan, renal recovery
Received
17 July 2025
Accepted
17 February 2026
Copyright
© 2026 Alrasheed, Alanazi, Bukhari, Albatati, Faqeehi and Alzabali. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
*Correspondence: Saeed Alzabali
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