Abstract: TH-PO957
Recurrent C3 Glomerulonephritis
Session Information
- Glomerular Trainee Case Reports
November 07, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Khan, Hameeda, Saint Barnabas Medical Center, West Orange, New Jersey, United States
- Liaqat, Aimen, Saint Barnabas Medical Center, West Orange, New Jersey, United States
Introduction
C3 Glomerulopathy encompasses dense deposit disease and C3 glomerulonephritis. On electron microscopy it is defined as C3 deposits more than 2 orders of other immunoglubulins. Pathogenesis involves activation of alternative complement pathway due to genetic or acquired mutations. Post infectious glomerulonephritis with persistent hematuria or proteinuria is termed as atypical. We present a case of C3 glomerulonephritis presenting as post infectious glomerulonephritis then crescenteric C3 treated with immunosuppression.
Case Description
A 54 year old female with history of glomerulonephritis (2008, 2017) presented with hemtaturia. Labs significant for creatinine 3mg/dl (baseline1.7), protein excretion 2g/day, C3 9mg/dl and C4 20mg/dl. Urine analysis with 4000 red blood cells. Workup for acquired factors showed normal factor H, serum C5b-9, negative C3 nephritic factor and CH50 less than 10mg/dl. Urine and blood cultures, antibodies to Proteinase-3, myeloperoxidase and genetic testing was negative. Intravenous steriods were given twice daily for 4 days, transitioned to prednisone. Due to increased creatinine hemodialysis was initiated. Biopsy showed crescenteric C3 dominant proliferative glomerulonephritis. Immunofluorescence 3+C3, 2+kappa and 1+lambda. Electron microscopy showed scattered subepithelial hump-like intermembranous and mesangial deposits. After 6 doses of cyclophosphamide 750mg, in 3 months renal function stabilized with creatinine 1.34mg/dl, urine protein excretion 0.8g and C3 153mg/dl.
In 2008, kidney biopsy showed post-infectious glomerulonephritis, low C3 and CH50 responsive to steroids. In 2016 she presented with pyelonephritis, creatinine 2.8mg/dl, protein excretion 2 g/day, urinalysis 3+ red blood cells, low C3 and normal C4. Biopsy showed membranoproliferative glomerulonephritis with crescents. Immunofloresence positive for IgG 2+, C3 2+, kappa 1+ and lambda 2+. Creatinine and C3 levels normalized post steriod therapy.
Discussion
Atypical Post infectious glomerulonephritis and C3 glomerulopathy are two sides of the same coin involving alternative complement pathway activation as shown by our patient’s biopsy findings. Due to repetitive steriod and immunosuppression responsiveness we conclude that recurrent cases of atypical post infectious glomerulonephritis be investigated for alternate pathway mutations. Novel anticomplement medications such as eculizumab may also be used.