Abstract: PO1556
Remission of C3 Glomerulonephritis with Rituximab
Session Information
- Glomerular Diseases: Clinical Features and Outcomes in Nephrotic Syndromes and Complement-Mediated Diseases
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Angamuthu, Akilandanayaki, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
- Batool, Fatima, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
- Gopisetti, Neethu, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
- Dissanayake, Imara, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
- Shin, Ji Young, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
Introduction
C3 glomerulonephritis (C3GN) is rare form of glomerulonephritis. It is diagnosed primarily by kidney biopsy with immunofluoroscence showing deposits of C3 along the basement membranes. It differs from dense deposit disease with absence of the pathognomonic deposits on electron microscopy (1). There are no radomized trials to guide therapeutic decisions. Spontaneous and treatment-associated complete remissions are rare.
Case Description
69-year-old female with history of well controlled hypertension on amlodipine was sent to hospital with elevated creatinine of 2.52 (unknown baseline) with positive ANA - 1:320 titer. Patient was asymptomatic with negative family history of kidney disease. She denied taking NSAIDs. Serum creatinine on admission was 3.05 mg/dl. Urinalysis showed numerous RBCs with 2 g of proteinuria in the 24-hour urine collection. P- ANCA was positive. All other pertinent serologies were negative including complements, C- ANCA, MPO, PR3. Renal ultrasound revealed medical renal disease. Renal biopsy was performed and preliminary reports showed crescentic glomerulonephritis. Pulse dose steroids was started followed by oral prednisone. In addition, Rituximab was started weekly. Final biopsy confirmed C3GN. Patient did not require dialysis and renal function significantly improved after 4 doses of rituximab to serum creatinine of 1.5 mg/dl.
Discussion
In C3GN with rapidly progressive glomerulonephritis (crescents on biopsy), treatment is not well established. Most patients are treated with steroids in combination with either cyclophosphamide or Mycophenolate mofetil (2). Rituximab was used in some case reports (3). In our patient, since P-ANCA was positive and preliminary biopsy showed crescents, Rituximab was started immediately after pulse dose steroids. Patient did not require dialysis, due to good response and remission.
Rituximab is a promising treatment option for C3GN.
Reference
Smith, R.J.H., et al C3 glomerulopathy — understanding a rare complement-driven renal disease. Nat Rev Nephrol 15, 129–143 (2019).
Fernando Caravaca-Fontán et al. Mycophenolate Mofetil in C3 Glomerulopathy and Pathogenic Drivers of the Disease. Clin J Am Soc Nephrol. September 2020, 15 (9) 1287-1298.
Giaime P et al. Remission of C3 glomerulopathy with rituximab as only immunosuppressive therapy. Clin Nephrol. 2015 Jan; 83(1):57-60.