Abstract: SA-PO730
The Maximal Consequences of Minimal Change Disease
Session Information
- Glomerular Diseases: Clinical, Outcomes, Trials - III
November 05, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials
Authors
- Dipollina, Chris, University of Connecticut, Storrs, Connecticut, United States
- Bustos, Brian, University of Connecticut, Storrs, Connecticut, United States
- Kae, Soo Hyun, University of Connecticut, Storrs, Connecticut, United States
Introduction
Minimal change disease (MCD) is a common cause of nephrotic syndrome in children. However, it only accounts for 10-15% of nephrotic syndromes in adults. The exact pathophysiology of MCD still remains unclear. We present a case of adult-onset MCD causing nephrotic syndrome and explore the pathophysiology behind MCD.
Case Description
A 38-year-old male presented to the hospital with 2 weeks of lower extremity edema and 3 days of oliguria with outpatient blood work showing creatinine rise to 1.5mg/dL from a baseline of 0.7 mg/dL 4 months ago. He had gross anasarca on exam, hypoalbuminemia with albumin level of 2.0 g/dL, and significant proteinuria (>7g/24 hrs) consistent with nephrotic syndrome. He denied being started on any new medication or using illicit drugs. During admission, his creatinine rose to 2.6mg/dL and he also had hematuria on urinalysis prompting a renal biopsy, which showed findings consistent with acute tubular necrosis (ATN) and diffuse podocytopathy confirming diagnosis of MCD with concurrent ATN. ANA, ANCA, and anti-PLA2R were all negative. Patient was pulsed with IV steroids and discharged on a steroid taper with resolution of his edema and proteinuria and normalization of his creatinine.
Discussion
The hallmark of MCD is podocyte effacement that results in protein loss, especially albumin, subsequently causing edema and hyperlipidemia. The exact mechanism as to how the podocyte gets effaced remains unclear although there are some emerging hypotheses. One theory is that T cell dysfunction results in production of glomerular permeability factor, which is thought to affect the glomerular capillary wall and cause proteinuria and foot effacement. More recent studies have shown the presence of deposits of circulating autoantibodies targeting nephrin, an essential component of the glomerular slit diaphragm, in kidney biopsies of patients with MCD. These hypotheses point towards an autoimmune or immune dysregulatory mechanism behind MCD, which can potentially explain why patients with MCD respond to immunosuppressives agents. Although rarer in adults, MCD is a known cause of nephrotic syndrome and carries significant morbidity with nearly 20% of cases necessitating dialysis. Kidney biopsy is the only way to confirm diagnosis. Further studies are needed to identify serological markers that could aid in a more prompt diagnosis of MCD in order to pursue targeted management.