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Abstract: SA-PO801

Long-Term Safety and Efficacy of Pegcetacoplan in Patients with C3 Glomerulopathy or Primary Immune-Complex Membranoproliferative Glomerulonephritis: The Long-Term VALE Extension Study

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Nester, Carla M., University of Iowa, Stead Family Children’s Hospital, Iowa City, Iowa, United States
  • Ariceta Iraola, María Gema, Universitat Autonoma de Barcelona, Barcelona, Catalunya, Spain
  • Mukherjee, Anwesha, Apellis Pharmaceuticals Inc, Waltham, Massachusetts, United States
  • Li, Li, Apellis Pharmaceuticals Inc, Waltham, Massachusetts, United States
  • Szamosi, Johan, Swedish Orphan Biovitrum AB, Stockholm, Stockholm, Sweden
  • López Lázaro, Luis, Swedish Orphan Biovitrum AB, Stockholm, Stockholm, Sweden
Background

C3 glomerulopathy (C3G) and primary immune-complex membranoproliferative glomerulonephritis (IC-MPGN) are rare diseases caused by complement overactivation leading to excessive deposition of C3 breakdown products in the kidney, causing inflammation, renal damage, and renal failure. Pegcetacoplan (PEG; C3/C3b inhibitor) blocks overactivation of alternative and classical complement pathway by inhibiting both C3 and C5 convertases (Figure). In the phase 2 trials, PEG demonstrated clinical benefit and was well tolerated. The Phase 3 VALIANT (NCT05067127) trial, in patients with C3G or primary IC-MPGN in native kidney or post-transplant, will further evaluate the safety and efficacy of PEG. Here we describe VALE (NCT05809531), a phase 3 extension study designed to evaluate long-term efficacy and safety of PEG in C3G and primary IC-MPGN.

Methods

Patients ≥12 years old who completed VALIANT through week 52 and achieved clinical benefit will continue to receive twice-weekly doses of PEG for a minimum of approximately 2.5 years. The primary efficacy endpoint is the log-transformed urine protein-to-creatinine ratio vs baseline. Secondary endpoints will evaluate changes in estimated glomerular filtration rate, proteinuria, and patient-reported outcomes, and disease progression defined by a composite clinical outcome. Safety endpoints will assess treatment-emergent adverse events.

Results

Not available.

Conclusion

The VALE extension will assess long-term efficacy and safety of PEG in C3G and primary IC-MPGN.