Abstract: TH-PO504
Clinical and Histologic Profile of C3 Glomerulopathy: A Retrospective Study
Session Information
- Glomerular Diseases: Clinical, Outcomes, Trials - I
November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials
Authors
- Bagchi, Soumita, Nephrology Department, All India Institute of Medical Sciences, New Delhi, India
- Barwad, Adarsh, Pathology Department, All India Institute of Medical Sciences, New Delhi, India
- Singh, Geetika, Pathology Department, All India Institute of Medical Sciences, New Delhi, India
- Subbiah, Arunkumar, Nephrology Department, All India Institute of Medical Sciences, New Delhi, India
- Bhowmik, Dipankar M., Nephrology Department, All India Institute of Medical Sciences, New Delhi, India
- Agarwal, Sanjay K., Nephrology Department, All India Institute of Medical Sciences, New Delhi, India
Background
C3 glomerulopathy(C3G) is a type of glomerular disease characterized by the predominant deposition of complement component C3 in the glomeruli due to the abnormal activation of the alternate complement pathway. Our understanding of the disease is still evolving. We evaluated the clinical profile of patients with C3G in our renal biopsy cohort.
Methods
Patients with biopsy proven C3G diagnosed between 2015 to 2020 were included in this retrospective study. Demographic characteristics, laboratory investigations and treatment details were obtained from medical records. Biopsy records were reviewed for histologic features. Complete remission (CR) was defined as 24-hour urine protein of <500 mg/d with stable eGFR and partial remission (PR) was defined as atleast 50% reduction in proteinuria with stable eGFR with a decline of proteinuria to <3.5g/day.
The study was approved by the institute ethics committee with a waiver of consent. Renal progression was defined as sustained >50% decline in eGFR or end stage renal failure.
Results
33 patients were diagnosed with C3G during the study period. Median age at presentation was 23(IQR 18-30) years and 69.7% were males. 51.5% had hypertension. Median estimated glomerular filtration rate(eGFR) was 49(IQR 22.5-97.8) ml/min/173m2, median serum albumin was 2.7(IQR 2.3-3.5) g/dl and median proteinuria was 4.3(IQR 3.0-7.4) g/day at presentation.51.5% patients had microscopic hematuria. 51.4% cases had low C3 levels. On histology 4 patients had dense deposit disease (DDD) while 29 were diagnosed with C3 glomerulonephritis (2 cases had crescents). 52.9% cases received renin angiotensin system (RAS) blockade therapy.
29 patients received initial immunosuppression with: steroids (8), mycophenolate mofetil+steroids (20) or cyclophosphamide+steroids (1). Only 9 patients achieved reduction in proteinuria with stable eGFR:5 had partial remission and 4 had complete remission. Of the 20 patients who received MMF, only 7(35%) had remission. During a median follow up of 29 (IQR 6.7 to 44.1) months, 15(45.4%) patients had sustained >50% decline in eGFR or had progressed to end stage renal failure.
Conclusion
C3G presents at a young with significant proteinuria. Patients respond poorly to currently available immunosuppression with high risk of worsening kidney function.