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Abstract: TH-PO677

ANCA-Negative Vasculitis Leading to Intrauterine Fetal Demise

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Bhatt, Dhirisha, The University of Tennessee Health Science Center College of Medicine, Memphis, Tennessee, United States
  • Amin, Sahar, The University of Tennessee Health Science Center College of Medicine, Memphis, Tennessee, United States
  • Wall, Barry M., VA Memphis Medical Center, Memphis, Tennessee, United States
  • Chedid, Alice, The University of Tennessee Health Science Center College of Medicine, Memphis, Tennessee, United States
Introduction

ANCA-negative pauci immune crescentic glomerulonephritis is a relatively rare subtype of RPGN, accounting for ~10-30% of cases. ANCA-negative vasculitis complicating a pregnancy course is even more rare. We present an interesting case where fetal demise at 20 weeks was the first manifestation of ANCA vasculitis.

Case Description

A previously healthy 16-year-old, G1P0 at 20 weeks of gestation, presented with vaginal bleeding and lower limb edema of 3-4 days duration. No further PMH except a recently completed antibiotic course 3 weeks ago for a URI.
She was afebrile, HR 116/min, BP 158/110mmHg, SpO2 97% on RA. Lab workup WBC 8.4, Hgb 7.0, platelets 207, Na 136, K 4.4,
HCO3 17, BUN 76, Cr 9.4 mg/dl (no baseline), and albumin 2.6 g/dl. UA: microscopic hematuria, 3+ protein. UPCR 6.3 g/g. UDS negative. Autoimmune workup was all negative including PR3/MPO. Trans-abdominal US showed fetal demise for which she underwent emergent vaginal delivery. From a kidney standpoint, the patient required hemodialysis on admission for volume overload. Given her severe anuric AKI, she underwent a kidney biopsy that showed: Diffuse necrotizing and crescentic glomerulonephritis, pauci immune,23/24 glomeruli with cellular crescents and fibrinoid necrosis. The patient was treated with pulse steroids, rituximab 1 gram x 2, and plasmapheresis every other day for total of 7 sessions. She became non-oliguric; however, remained dialysis-dependent at the time of transfer to a pediatric hospital.

Discussion

We present a challenging case of ANCA-negative vasculitis complicating pregnancy leading to fetal demise. Our patient was managed similarly to an ANCA-positive vasculitis. We wonder if adding aserum creatinine to the routine urinalysis as part of first-trimester screening would have led to earlier diagnosis and changed this unfortunate outcome.

Cellular crescents