Abstract: SA-PO812
C3 Glomerulopathy: A Pediatric and Adult Case Series in New Mexico
Session Information
- C3G, TMA, MGRS, Amyloidosis, and More
October 26, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Avila, Paul Brandon, Universidad de San Carlos de Guatemala, Ciudad de Guatemala, Guatemala, Guatemala
- Singh, Namita, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, United States
- Staples, Amy, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, United States
- Schmidt, Darren W., University of New Mexico Health Sciences Center, Albuquerque, New Mexico, United States
- Garcia, Pablo, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, United States
Introduction
C3 glomerulopathy (C3GN) results from alternative complement pathway dysregulation. C3G manifests with proteinuria, decreased kidney function, and low serum C3 levels. Therapeutic options are limited. Improving diagnostic criteria and treatment strategies is essential for better outcomes in C3GN. Here, we report a case series of five cases in our pediatric and young adult population of C3GN with long term follow up in New Mexico.
Case Description
Five patients, aged 8 to 15 years, were diagnosed with C3GN based on kidney biopsy findings or clinical presentation and genetics. All were treated with C5 inhibitor therapy (C5IT), with two receiving additional immunosuppressive therapy (IST) and three undergoing PLEX therapy. Details of each case are summarized in table 1. During C5IT treatment lasting 1 to 9 years, 4 patients showed kidney function improvement. Three patients experienced reduced proteinuria (urine protein-to-creatinine ratio, UPCR), with those initially presenting with lower eGFR and higher C3 levels responding better. Two patients achieved complete remission (UPCR <0.3 g/g), and four showed significant improvement in eGFR post-treatment.
Discussion
In this case series, C3G treated with C5IT showed positive results regarding kidney function. The effect of C5IT on proteinuria reduction was not clear. The role of PLEX in pediatric cases is promising. Two achieved complete remission (UPCR <0.3 g/g). These findings support C5IT as a promising therapy for C3GN. Larger multicenter studies in pediatric and young adult populations are needed. Furthermore, novel therapies are needed to improve outcomes as the degree of proteinuria could correlate with disease activity.
Clinical characteristics, treatment, and outcomes of followed patients diagnosed with C3GN