Abstract: PUB549
A Rare Case of Fibrillary Glomerulonephritis and Advanced Diabetic Glomerulosclerosis Class IIb in a Patient with Newly Diagnosed Nephrotic Syndrome
Session Information
Category: Trainee Case Report
- 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix
Authors
- Obadan, Odianosen Idiahi, Rutgers New Jersey Medical School, Newark, New Jersey, United States
- Mahendrakar, Smita, Rutgers New Jersey Medical School, Newark, New Jersey, United States
Introduction
Fibrillary glomerulonephritis is a rare and underdiagnosed glomerular disorder. It was first described by Rosenmann and Eliakim in 1977. It is defined by the ultrastructural finding of haphazardly arranged, straight fibrils measuring 10 to 30 nm in thickness. According to literature, it coexists in about 20% of Type 2 diabetic patients but the connection between these is a topic of debate.
We present a case of fibrillary glomerulonephritis coexisting with diabetic nephropathy in a type 2 diabetic with newly diagnosed Nephrotic syndrome.
Case Description
A 48 years old female with past medical history of CHF, polysubstance use, CKD and hypertension, who presented for follow up at renal clinic following discharge from the hospital. She had presented to the emergency room with uncontrolled blood pressure (245/112mmHg), acute kidney injury and nephrotic syndrome, physical examination generated 2+ pitting pedal edema. Creatinine level was 4.1 mg//dl, eGFR of 14, urinary protein excretion was 3.1g/day , total cholesterol of 229 mg/dL, LDL was 123mg/dL and albumin of 3.1g/dL, Kappa/Lambda ratio of 2.56, SPEP had positive polyclonal gammopathy.
The patient’s kidney biopsy showed fibrillary glomerulonephritis and diabetic nephropathy, moderately advanced, diffused diabetic glomerulosclerosis, interstitial fibrosis and tubular atrophy, severe arteriosclerosis and moderate arteriolosclerosis.
She was offered treatment for high blood pressure with lisinopril, amlodipine, labetalol, clonidine and statins.
Discussion
Reports have shown that fibrillary Glomerulonephritis can coexist with diabetic nephropathy, but a true connection has not been proven. Accelerated glycosylation of proteins in diabetics and advanced glycosylation end products capable of cross-linking with other structural proteins as well as circulating proteins has been suggested as a possible pathophysiology. There is currently no effective therapy as most therapies are guided towards reducing proteinuria and cholesterolemia. Cytotoxic agents, plasmapheresis are used but are not ideal therapies and more studies need to be performed to determine the link between diabetic nephropathy and fibrillary Glomerulonphritis in order to determine the best or optimal therapy.