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Kidney Week

Abstract: TH-PO524

Podocytic Infolding Glomerulopathy in a Patient with Systemic Lupus Erythematous

Session Information

  • Trainee Case Reports - I
    October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Chedid, Alice, Johns Hopkins University School of Medicine, Division of Nehrology, Baltimore, Maryland, United States
  • Hanouneh, Mohamad A., Johns Hopkins University School of Medicine, Division of Nehrology, Baltimore, Maryland, United States
  • McMahon, Blaithin A., Johns Hopkins University School of Medicine, Division of Nehrology, Baltimore, Maryland, United States
Introduction

Podocytic infolding glomerulopathy (PIG) has been proposed as a new disease entity. This is first case of PIG reported in a woman patient of African American ancestry. Five case reports of PIG complicated by focal segmental glomerulosclerosis (FSGS).

Case Description

A 35-year-old African American woman with a history of systemic lupus erythematous and autoimmune enteritic ganglionitis presents with new acute kidney injury with serum creatinine 1.6 mg/dL (baseline 0.8 mg/dL) and nephrotic range proteinuria. Urinalysis was positive for 3 red blood cells, 5 white blood cells per high power field and no red blood cell casts. Urine protein/creatinine ratio was 19 g/g. C3 and C4 levels were 90 and 30 mg/dL, respectively. Anti-DNA screen, anti-PLA2 receptor antibody, HIV and hepatitis panel was negative. Renal sonogram showed normal sized kidneys with minimally increased echogenicity and unchanged mild bilateral hydronephrosis.

Kidney biopsy demonstrated significant FSGS with collapsing features. Under periodic acid methenamine silver (PAMS) staining, the glomeruli had a diffuse moth-eaten appearance along glomerular capillary loops (Figure A). On ultrastructural examination there were numerous electron dense intramembranous membrane bound structures and microspheres entrapped in the glomerular basement membrane (GBM) of capillary loops (Figure B and Inset) consistent with PIG. Despite treatment with various immunosuppressive therapies the patient still progressed to end stage renal disease.

Discussion

In PIG, microspheres or microtubular structures, or both, are associated with the infolding of cytoplasmic processes of podocytes into the GBM as a consequence of capillary wall remodeling. The mechanism of PIG is unknown and has been reported in predominantly in Asian and in the setting of connective tissue diseases.