J Nephropathol. 2019;8(3): e28.
doi: 10.15171/jnp.2019.28
  Abstract View: 317
  PDF Download: 147

Case Report

A rare case of focal segmental glomerular sclerosis and subsequent necrotizing crescentic glomerulonephritis in the same patient

Jake Cho 1,2 * ORCiD, Erika Lara 1,2, Izuchukwu Nwakoby 3,4

1 University of Central Florida College of Medicine, Graduate Medical Education, Orlando, Florida, USA
2 Ocala Regional Medical Center, Internal Medicine Residency Program, Ocala, Florida, USA
3 Ocala Regional Medical Center, Nephrology Department, Ocala, Florida, USA
4 The Ocala Kidney Group, Ocala, Florida, USA

Abstract

Background: Focal segmental glomerular sclerosis (FSGS) and necrotizing crescentic glomerulonephritis is a rare combination of diagnoses in the same patient. We report on a patient with FSGS who 10 years later developed anti-neutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis.

Case Presentation: Patient is a 60-year-old female with chronic kidney disease stage 3, osteopenia and anemia. In 2007, she was positive for ANCA proteinase-3 antibody, but kidney biopsy revealed FSGS. She was treated with high-dose oral steroids with tapered dose and went into remission. In 2017, she developed acute renal failure with increased proteinuria. Despite prior FSGS diagnosis, her new kidney biopsy revealed pauci-immune necrotizing glomerulonephritis. Patient was treated with methylprednisolone 250 mg IV for three days and high dose oral steroids with tapered dose. She was also started on rituximab 375 mg/m2 IV once weekly for 4 doses. Given the extent of kidney damage, the patient decided to start peritoneal dialysis and she is also on the kidney transplant list.

Conclusions: The rare concurrence of FSGS and ANCA associated glomerulonephritis has not yet been reported. The case also emphasizes the significance of screening for ANCA or obtaining kidney biopsy when indicated not only as the gold standard for diagnosis but also as prognostic value.

Implication for health policy/practice/research/medical education:

This case presents a first reported occurrence of dual glomerulopathy consisting of focal segmental glomerular sclerosis and ANCA-associated vasculitis. Although both are rare conditions, if dual glomerulopathy is suspected in the setting of acute renal failure with an active urine sediment, then pauci-immune crescentic glomerulonephritis should be considered in the differential diagnosis. The clinical significance of “double positive” renal disease is that it often portends worse prognosis and more challenging treatment. Workup should include screening for ANCA as well as obtaining renal biopsy to avoid diagnostic anchoring bias and obtain an accurate diagnosis to begin timely treatment and preserve renal function.

Please cite this paper as: Cho J, Lara E, Nwakoby I. A rare case of focal segmental glomerular sclerosis and subsequent necrotizing crescentic glomerulonephritis in the same patient. J Nephropathol. 2019;8(3):e28. DOI: 10.15171/jnp.2019.28.

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Submitted: 18 May 2018
Accepted: 03 Jul 2019
First published online: 25 Jul 2019
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