Abstract: PO1861
Fibrillary Glomerulonephritis Treated with Rituximab: A Case Report
Session Information
- Glomerular Diseases: Clinical, Outcomes, and Trials - 1
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Laquindanum, Serah Kae Labitad, Makati Medical Center, Makati City, Philippines
- Penaranda, Eladio Miguel M., Makati Medical Center, Makati City, Philippines
Introduction
Fibrillary Glomerulonephritis (FGN) is a rare primary glomerular disease first described by Rosenmann and Eliakim in 1977 present in 0.5 to 1% of native kidney biopsies defined by haphazardly arranged fibrils 10 to 30 nm in thickness deposited in the mesangium, glomerular basement membranes or both. Initially, FGN was considered to be idiopathic. However, approximately one-third have a history of malignancy, monoclonal gammopathy, autoimmune disease, hepatitis C infection or an IgM glomerular deposit disease. Prognosis is generally poor with 50% of patients developing ESRD within 6 years of presentation. The most common form of treatment is steroids with or without a second agent usually Cyclophosphamide or Rituximab. To date, there is no convincingly effective treatment but published case series reports clinical response referred to as “nonprogression” defined by stable renal function in those treated with Rituximab.
Case Description
Our patient is a 49-year old Filipino female, hypertensive, diagnosed case of Immune Complex-Mediated Glomerulonephritis presenting with elevated blood pressure and nephrotic-range proteinuria. Initial adjustment of her anti-hypertensive regimen controlled her blood pressure. Subsequently, she developed resistant hypertension and increasing proteinuria. Creatinine increased to 2.1 mg/dL from a baseline of 1 mg/dL in 12 months. A second renal biopsy was done showing fairly widespread podocyte foot process effacement and mesangial fibrillary deposits measuring 13 nm in mean diameter suggestive of Fibirillary Glomerulonephritis. Patient was worked up for an underlying malignancy, autoimmune disease and infectious causes but none turned out positive. At this time, proteinuria has increased to 7596.05 grams from 1622.08 grams with a creatinine clearance of 39.80 ml/min/1.73 m2. After discussing with the patient, she was given Rituximab as four weekly doses of 375 mg/m2 intravenously. After five months, there was significant reduction in proteinuria at 1734.32 grams with stable creatinine clearance of 31.99 ml/min/1.73 m2.
Discussion
In general, FGN prognosis is poor and majority of patients progress to ESRD. Treatment options are currently limited and conclusions regarding immunosuppressive therapy cannot be drawn from limited published data. Rituximab may offer benefit particularly in patients with relatively normal baseline renal function.