Abstract: PO1673
Nephrotic Syndrome in a Patient with Systemic Lupus Erythematous: Is it Lupus Podocytopathy?
Session Information
- Podocyte Injury in Human Disease: Pathomechanism, Diagnosis, and Therapy
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1204 Podocyte Biology
Authors
- Hernandez Garcilazo, Nora H., Michigan State University, East Lansing, Michigan, United States
- Hassanein, Mohamed, Cleveland Clinic, Cleveland, Ohio, United States
- Garces, Christopher Cantoria, Michigan State University, East Lansing, Michigan, United States
- Khor, Si Yuan, Michigan State University, East Lansing, Michigan, United States
- Kaw, Beenu, Sparrow Health System, Lansing, Michigan, United States
Introduction
Lupus podocytopathy (LP) is a rare form of lupus nephritis (LN) that clinically mimics minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS). We present a case of LP in a patient with systemic lupus erythematosus (SLE).
Case Description
A 73-year-old female with a history of SLE (on Hydroxychloroquine), and nephrolithiasis presented with left flank pain for 2 weeks. Physical exam was notable for peripheral edema and left costovertebral angle tenderness. Labs showed a creatinine 1.02 mg/dL (baseline 0.7), serum albumin 2.6 mg/dL, and urine microalbumin/creatinine (MA/Cr) ratio of 7.5 g/g. Further workup revealed a positive ANA, negative anti-dsDNA antibodies, and normal complements. Kidney biopsy showed FSGS, tip variant, and 100% podocyte foot process effacement (FPE). She was started on high-dose prednisone, simvastatin, ACE inhibitor, and furosemide with potassium supplementation for significant pedal edema. Two months later, her MA/Cr ratio improved to 3 g/g. Prednisone was slowly tapered off over a 7-month period during which symptoms were well-controlled and MA/Cr ratio continued to improve.
Discussion
Nephrotic syndrome in patients with SLE raises suspicion for LP, which represents 1% of LN biopsies. Biopsy findings include diffuse FPE (>70%) on electron microscopy, and absence of immune deposits on light, immunofluorescence, and electron microscopy. LP is divided into MCD or FSGS, with the latter having higher rates of hypertension, acute kidney injury on presentation, and overall worse outcomes. Treatment consists of a short course of high-dose glucocorticoids; however high rates of relapse are observed and tend to coincide with SLE activity.
A. H&E of a glomerulus showing focal segmental glomerulosclerosis, with segmental scars near the tip of the glomerular tuft. There is mild mesangial matrix expansion.
B. Electron microscopy showing diffuse podocyte foot process effacement with no mesangial or glomerular basement membrane immune-type electron-dense deposits identified.