ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

Abstract: FR-PO0911

Clinical Variability and Outcomes in C3 Glomerulopathy: A Single-Center Experience

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Khullar, Dinesh, Max Super Speciality Hospital Saket, New Delhi, Delhi, India
  • Singh, Abhishek, Max Super Speciality Hospital Saket, New Delhi, Delhi, India
  • Singh, Kulwant, Max Super Speciality Hospital Saket, New Delhi, Delhi, India
  • Kumar, Deepak, Max Super Speciality Hospital Saket, New Delhi, Delhi, India
  • Grover, Rahul, Max Super Speciality Hospital Saket, New Delhi, Delhi, India
  • Singh, Prof Narinder Pal, Max Super Speciality Hospital Saket, New Delhi, Delhi, India
  • Sawhney, Simran, Max Super Speciality Hospital Saket, New Delhi, Delhi, India
  • Gupta, Anish Kumar, Max Super Speciality Hospital Saket, New Delhi, Delhi, India
  • Chavan, Abhijit Suresh, Max Super Speciality Hospital Saket, New Delhi, Delhi, India
Introduction

C3 glomerulopathy (C3G) is a rare disease with variable presentations ranging from asymptomatic isolated microscopic hematuria(21-36%), acute nephritis(16-38%), nephrotic range proteinuria(15-41%), frank nephrotic syndrome(12-55%) and AKI(8%). Unfortunately, once diagnosed with C3G, patients have a varied course with progression to ESKD (50% by 10 years) and need renal replacement therapy at a very young age. The treatment options include conservative management of proteinuria with RAAS blockade, and immunosuppressive therapy.

Case Description

We present four cases of biopsy-proven C3G. Electron Microscopy was not done in all cases. Secondary causes of C3G had been excluded. Case 1 had a family history with membranoproliferative glomerulonephritis present in 2 generations and all siblings were affected with C3G. Case 2 had presented with AKI with crescents in kidney biopsy along with moderate Interstitial fibrosis and tubular atrophy. Case 3 was initially diagnosed and treated as a case of childhood onset nephrotic syndrome and biopsied in view of steroid resistance, which revealed a collapsing pattern with C3 deposits. Her nephrotic syndrome has been remitting and relapsing with response and proteinuria reduction to cnis but with relapses. Case 4 underwent partial remission and was offered a clinical trial with investigational drug Iptacopan, which showed evident benefit in proteinuria reduction and stabilization of eGFR.

Discussion

The cases highlight the heterogeneity in presentation and progression of C3 glomerulopathy, ranging from isolated nephrotic syndrome to AKI with crescentic glomerulonephritis. Family history played a key role in Case 1, indicating a strong genetic predisposition. Case 2 progressed rapidly to ESKD despite immunosuppression, while Case 3 showed a relapsing-remitting course with steroid resistance. Case 4 showed partial remission following participation in a clinical trial, underscoring the potential role of emerging therapies in C3G management.

Digital Object Identifier (DOI)