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Kidney Week

Abstract: SA-PO593

A Unique Presentation of Dense Deposit Disease

Session Information

  • Pediatric Nephrology - II
    November 05, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1800 Pediatric Nephrology

Authors

  • Sadeghiani, Golnaz, Our Lady of the Lake Regional Medical Center, Baton Rouge, Louisiana, United States
  • Ezzaiyani, Amal G., Our Lady of the Lake Regional Medical Center, Baton Rouge, Louisiana, United States
  • Rawson, Ashley E., Our Lady of the Lake Regional Medical Center, Baton Rouge, Louisiana, United States
Introduction

Diagnosis of dense deposit disease (DDD) is made by renal bx. It has a poor prognosis and treatment remains difficult and varied. Renal bx may be confounding early in the disease process.

Case Description

A 14 yo female with no PMH presented to our facility with 4 weeks of progressive edema. Initial evaluation demonstrated UA with 20-50 WBCs, 10-20 RBCs, and >500 protein; serum Cr 1.54; UPCR 13000 mg/G; C3 11; and C4 11. ANA and anti-dsDNA antibodies were positive.
Renal bx demonstrated a diffuse proliferative glomerulonephritis (GN) and membranous nephropathy with multiple wire loop lesions. IF showed a full house pattern with diffuse global granular mesangial and capillary loop staining for IgG (3+), IgA (trace-1+), IgM (1+), C3 (3+), C1q (2+), kappa (3+) and lambda (2+). EM revealed occasional subepithelial and intramembranous electron-dense deposits along the basement membranes.
She was diagnosed with mixed class IV/V lupus nephritis and met ACR classification criteria for diagnosis of SLE.
From 4/2020-4/2021,she was treated with high dose pulse IV steroids, oral steroids, rituximab, mycophenolate mofetil, azathioprine, and cyclophosphamide. Despite aggressive treatment, her response was moderate at best and the C3 did not increase.
She underwent a second bx 12 months later due to lack of resolution of renal symptoms. IF on the second bx revealed focal and segmental granular mesangial and capillary loop staining for IgG (1+), kappa (trace) and lambda (trace) and diffuse global granular mesangial and capillary loop staining for C3 (3+). EM revealed extensive electron-dense transformation of the glomerular basement membranes and mesangium. These findings appeared more consistent with DDD.
Complement pathway assessment revealed elevated SC5B-9 Level (1,048 ng/ml), C3a (454.5 ng/ml), C5a (28.616 ng/ml), C3 Nephritic Factor (171.0 unit/ml) and complement Bb (3.790 mcg/ml). She was started on eculizumab with significant improvement over the last six months. Her Cr fell from 2.52 to 1.41 and UPCR fell from 5,513 mg/G to 2,117 mg/G.

Discussion

GN patients present similarly in many cases and some lupus indicators such as ANA may be nonspecific. This case shows the importance of repeating a renal bx in non-responsive lupus nephritis or GN patients in general as the initial biopsy may not tell the full story. In our case repeat biopsy revealed a new diagnosis leading to different treatment options.