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Abstract: SA-PO916

C3 Glomerulopathy Current Treatment Options and Real-World Management: Results from a Multi-Country Study

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Lafayette, Richard A., Stanford University School of Medicine, Stanford, California, United States
  • Pannagl, Katharina, Novartis Pharmaceuticals UK Ltd, London, United Kingdom
  • Ndife, Briana C., Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, United States
  • Smeets, Serge, Novartis Pharma AG, Basel, Basel-Stadt, Switzerland
  • Murphy, Kathleen, Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, United States
  • de Courcy, Jonathan, Adelphi Real World, Bollington, Cheshire East, United Kingdom
  • Libby, Susanna, Adelphi Real World, Bollington, Cheshire East, United Kingdom
  • Proudfoot, Clare, Novartis Pharma AG, Basel, Basel-Stadt, Switzerland
Background

Complement 3 glomerulopathy (C3G) is a rare kidney disease, with an estimated incidence of 1-2/million/year. C3G is associated with a high risk of disease progression, approximately 50% of patients reach kidney failure within 10 years of diagnosis. KDIGO guidelines recommend treating with renin-angiotensin-aldosterone system inhibitors (RAASi) and in some patients, corticosteroids (CS) or mycophenolate mofetil (MMF), or eculizumab. This analysis aimed to better understand the treatment of C3G in the US, Europe, and Asia.

Methods

Data were drawn from the Adelphi C3G Disease Specific Programme, a real-world cross-sectional survey of C3G-treating nephrologists in US, France, Germany, Italy, Spain, UK (EU5), China and Japan from August 2022 to April 2023. Nephrologists completed forms via online links for consecutive patients presenting with C3G. Forms included patients’ demographics, clinical characteristics and C3G treatments.

Results

111 nephrologists completed records for 385 C3G patients (US 100, EU5 189, China 60, Japan 36). 321 (83%) patients were receiving treatment at time of survey. Of these, median patient age was 41 years, and 60% were male. Median proteinuria was 1.3 g/day. 63% of patients had proteinuria ≥1 g/day (Table 1). 70% were receiving RAASi, 49% CS, 27% MMF, and 30% biologics.

Conclusion

C3G is a rapidly progressing disease with no approved therapy. Most patients in this study were treated with both conventional immunosuppressants and biologics frequently added to RAASi. Despite this, proteinuria remained high, in most patients ≥1 g/day. This highlights the need for targeted therapies to treat the root cause of C3G.

Table 1: Current therapy and proteinuria levels by region

Funding

  • Commercial Support – Novartis Pharmaceuticals Corporation