Abstract: PUB168
Fibrillary Glomerulonephritis Disease Natural History: Three Cases with Different Clinical, Pathological Features, and Outcomes
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Griveas, Ioannis, Noseleutiko Idryma Metochikou Tameiou Stratou, Athens, Attica, Greece
- Revela, Ioanna, Frontis Dialysis Center, Piraeus, Attika, Greece
Introduction
Fibrillary glomerulonephritis (FGN) is a rare and poorly understood kidney disease and guidance on diagnosis and management is scant. Its clinical heterogeneity and high rate of progression to end-stage renal disease (ESRD) still pose significant diagnostic and therapeutic challenges. The optimal therapeutical approach is not clear, since treatment outcomes vary across the globe.
We retrospectively reviewed the medical data of 3 patients diagnosed with biopsy-proven FGN. This case series aims to enhance awareness of FGN and focus on the need for further research in order to improve patient outcomes.
Case Description
We reviewed the clinical, histopathological, and therapeutic data of three patients (2 women, one man, mean age at diagnosis: 60 years old) with FGN diagnosed by kidney biopsy. Οne lady of 75 years old was presented with hematuria, nephrotic syndrome ( 4 grs/24 hours) and normal renal function. Light microscopy showed mesangial proliferative , membranoproliferative-like, , and diffuse sclerosing patterns. Another lady of 49 years old was also presented with hematuria, nephrotic syndrome (7 grs/ 24 hours) but impaired renal function (creat: 2,5 mg/dl). Light microscopy showed partial hardening and certain fibrocytic meniscus formations. Our last patient of 55, was also presented with hematuria, nephrotic syndrome (4,2 grs/ 24 hours) and impaired renal function (creat: 1,5 mg/dl). Light microscopy showed mesangial proliferative , solidification, and diffuse sclerosing patterns. Diagnosis in our group of patients confirmed with the integration of DNAJB9 staining in kidney biopsy. Rituximab was used in all of our patients combined with steroids. First lady showed partial remission after 3 months of treatment but passed away due a surgical emergency. Our second lady developed rapidly progressive glomerulonephritis (RPGN) despite aggressive therapy and ended up to ESRD. Our last patient achieved partial remission (1grs proteinuria/24 hours, Creat: 1,2 mg/dl) and remains stable after 25 months of follow up.
Discussion
FGN is a heterogenous disease which still remains a diagnostic and therapeutic challenge. Further research into pathophysiological mechanisms and targeted therapies is essential to optimize management and outcomes for different kind affected patients. New treatment strategies seem to be a necessity.