Abstract: PUB394
A 30-Year-Old Caucasian Man with Phospholipase A2 Receptor-Associated Primary Membranous Nephropathy
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Aung, Htun Min, New York City Health and Hospitals Jacobi, Bronx, New York, United States
- Varma, Nidhi, New York City Health and Hospitals Jacobi, Bronx, New York, United States
Introduction
Primary membranous nephropathy (MN) is more commonly seen in Caucasian males over the age of 40 years. We present a unique case of anti-phospholipase A2 receptor (PLA2R)-associated primary MN in a 30-year-old Caucasian male who presented with end-stage kidney disease (ESKD).
Case Description
A 30-year-old Caucasian male patient with a history of chronic kidney disease stage 3B and obesity presented to the emergency department with fatigue, dizziness, and shortness of breath. History was notable for NSAID use a year ago that he had stopped. He denied illicit drug use or contrast exposure. He was not taking any medications at the time of presentation.
A chart review revealed that he was evaluated for serum creatinine (sCr) of 2.5 mg/dl and 1.5-gram proteinuria at a different hospital a year ago. Serologies with antinuclear antibody (Ab) and anti-double-stranded DNA Ab were negative. His CKD was thought to be secondary to NSAID use. But, he was lost to follow-up and presented to our hospital a year later with renal failure.
He was anuric with a blood pressure of 193/99 mmHg, sCr of 17.4 g/dL, bicarbonate 9.6 mEq/L, venous pH of 7.1, serum albumin of 4.2 g/dL, and creatine phosphokinase of 205 U/L. There was no urinary retention. He was emergently started on hemodialysis for uremic symptoms.
Urinalysis showed 4+ protein and RBCs of 13.6 per high-power field. The urine protein-creatinine ratio was 3,960 mg/gm. The right kidney was 12.8 cm, and the left was 13.9 cm on ultrasound. Renal artery stenosis was ruled out. Serological workup was negative except for the PLA2R Ab of 37. He underwent a kidney biopsy which revealed advanced membranous glomerulopathy stage 3 (PLA2R-positive), with extensive global and focal segmental sclerosing features, severe tubular atrophy and interstitial fibrosis with focal acute tubular injury, and severe arteriosclerosis and hyalinosis with focal intimal fibrin.
He was not a candidate for immunosuppressive therapy due to his poor renal prognosis. He was continued intermittent hemodialysis on discharge.
Discussion
Anti-PLA2R Ab is highly specific for the diagnosis of primary MN. In a young person with decreased GFR and proteinuria, PLA2R Ab should be considered to help with the diagnosis. Adding PLA2R Ab at the time of his first evaluation could have elucidated the cause of his chronic kidney disease and possibly prevented ESKD.