Abstract: PO1410
Fibrillary Glomerulonephritis: A Rare Entity, Responsive to Rituximab
Session Information
- Glomerular Diseases: Fibrosis and Extracellular Matrix
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix
Authors
- Bachu, Ramya, Baptist Health, Little Rock, Arkansas, United States
- Sarwar, Mujtaba, Baptist Health, Little Rock, Arkansas, United States
- Khan, Jahanzeb, Baptist Health, Little Rock, Arkansas, United States
- Bukhari, Sehrish W., Baptist Health, Little Rock, Arkansas, United States
- Syed, Anum, Baptist Health, Little Rock, Arkansas, United States
- Mousa, Heba, Baptist Health, Little Rock, Arkansas, United States
- Ijaz, Fakhar, Baptist Health, Little Rock, Arkansas, United States
Introduction
Fibrillary glomerulopathy (FGN) is a rare proliferative type of glomerular disease with poor
prognosis and limited therapeutic options. Literature review showed very few cases of FGN
where Rituximab has been used.
Case Description
A 69-year-old Caucasian woman with a history of DM, HTN presented to the hospital with
complaints of dyspnea and leg swelling. Lab work revealed BUN/Scr of 34/3.88 mg/dL (baseline
Scr of 1.4 mg/dL, proteinuria (7,902 mg/dL). Renal biopsy was performed which showed
fibrillary deposits as shown in the picture. Hepatitis panel and work up of malignancy including
bone marrow biopsy was negative. She was started on Rituximab 1gm every 14 days for 2
doses. Pt initially required HD but with treatment her symptoms and Cr improved and was taken
off HD. Most recent Cr was1.8.
Discussion
The diagnosis of FGN can only be established with kidney biopsy. Most of the patients present
with renal insufficiency, nephrotic picture. Mostly idiopathic but can be associated with hepatitis
C, dysproteinemia, autoimmune diseases and to lesser extent malignancies. The prognosis is
very poor with very limited therapeutic options to date and optimal therapy remains to be
ascertained. Rituximab has been used in few cases in literature showing good clinical response.
Our patient tolerated and responded very well to Rituximab with significant improvement in
kidney function and hence we propose the use of Rituximab in FGN.