Abstract: TH-PO460
Membranous Nephropathy Over the Counter? NELL Yes!
Session Information
- Glomerular Diseases: Podocytopathies and Nephrotic Syndromes
November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1302 Glomerular Diseases: Immunology and Inflammation
Authors
- Ali, Umair, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
- Gokden, Neriman, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
- Arthur, John M., University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
- Singh, Manisha, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
Introduction
Membranous nephropathy (MN) is a common cause of nephrotic syndrome.An autoimmune response to M-type phospholipase-A2-receptor (PLA2R) is usually associated with primary MN while antibodies like Neural-epidermal growth-factor-like-1 (NELL1) are related to secondary causes like malignancy or Lipoic acid (LA) ingestion. We present a case of MN with over-the-counter supplement use.
Case Description
A 65 year old man presented to hospital with lower limb swelling associated with a rash with history of diabetes, hypertension and benign prostatic hyperplasia. Examination showed bilateral pitting edema to lower limbs with a purpuric rash. Lab work showed HbA1c of 12.3%, serum albumin at 1.4 mg/dl,serum creatinine of 2.4 mg/dl. Urinalysis showed protein +3 and RBC +1 with 24-hour urine protein of 21 grams. Serological workup was negative for autoimmune conditions and infections. Renal biopsy showed MN. Immunotyping was negative for PLA2R and THSD7A but positive for NELL1(figure shown). Age-appropriate malignancy screen was negative. Based on NELL-1 immunotyping, an extensive review of medications was done. He used NSAIDs intermittently and also over-the-counter multivitamins including lipoic acid 1800 mg. The over-the-counter medications were stopped. The patient was treated with IV methyl-prednisone 1 gm for three days, followed by tapering oral prednisone. However, due to severe exacerbation of hyperglycemia, we switched to a steroid-sparing regimen with rituximab and tacrolimus. His edema subsided at the one-month follow-up visit and proteinuria improved to 6 grams.
Discussion
Lipoic-acid supplements are considered antioxidants, insulin-mimetic, and are a frequently used over-the-counter medication. Studies show an association between NELL1 and LA ingestion, though the number of cases known is only a few. In our case, a search for supplement use was made after the biopsy results, especially the immunotyping, leading to a tailored treatment plan. Treatment options are limited. We used rituximab with tacrolimus for this patient with uncontrolled DM. Addressing the driver of the disease,optimizing patient-specific treatment, following proteinuria for assessing remission are the mainstays of treatment of secondary MN
Renal Biopsy with PAS,EM & Immunotyping (NELL1)