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Kidney Week

Abstract: PO2211

Erdheim-Chester Disease: A Rare Cause of Bilateral Renal Artery Stenosis and AKI

Session Information

  • Onco-Nephrology - 2
    October 22, 2020 | Location: On-Demand
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Report

  • 1500 Onco-Nephrology

Authors

  • Ng, Monica S., Kidney Health Service, Royal Brisbane and Women’s Hospital, Brisbane, Queensland, Australia
  • Mon, Saw Yu, Kidney Health Service, Royal Brisbane and Women’s Hospital, Brisbane, Queensland, Australia
  • O'Rourke, Rachael, Medical Imaging, Prince Charles Hospital, Brisbane, Queensland, Australia
  • Mott, Nigel, Medical Imaging, Royal Brisbane and Women’s Hospital, Brisbane, Queensland, Australia
  • Taheri, Touraj, Pathology Queensland, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia
  • Morris, Kirk, Department of Cancer Services, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia
  • Gois, Pedro Henrique Franca, Kidney Health Service, Royal Brisbane and Women’s Hospital, Brisbane, Queensland, Australia
Introduction

Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis affecting multiple organ systems. We report a case of ECD with bone marrow, cardiac and vascular and renal involvement, presenting with hypertensive emergency and AKI, responsive to endovascular stenting and peg-interferon.

Case Description

A 72 y.o. female presented with hypertensive emergency and AKI. Her symptoms included fatigue, poor oral intake, 5kg weight loss and lower limb oedema. Her medical history included stage 3b chronic kidney disease secondary to hypertensive nephropathy, bilateral renal artery stenosis, coronary artery disease, rheumatoid arthritis, previous diverticulitis and osteoarthritis. Her examination was unremarkable. Her investigations showed anaemia; creatinine 2.45mg/dL (baseline 1.59mg/dL), BUN 47mg/dL; unremarkable electrolytes and liver function tests. A CT angiogram showed extensive, irregular mural thickening of the aorta present for 2 years, right atrial mass, high-grade stenosis of both renal arteries and left vertebral artery occlusion. Review of prior lower limb CT imaging showed bilateral, symmetrical sclerotic bone infiltrates in the diaphyses and metaphyses, corresponding to the regions of PET avidity, confirming the diagnosis of ECD. Echocardiogram and cardiac MRI confirmed a 21.7 x 18.3mm right atrial mass with small pericardial effusion. ECD was confirmed via right sacrum lesion biopsy showing sclerotic xantogranulomatous histiocytic(CD68+, CD163+, CD1a-) reaction. Despite taking 5 classes (7 agents) of anti-hypertensives at maximal doses, her blood pressure was suboptimally controlled. After bilateral endovascular renal artery stenting, anti-hypertensive requirement reduced to 2 agents and renal function improved to better than pre-AKI levels. She is receiving peg-interferon α2a and prednisolone for ECD.

Discussion

The disease affects renal arteries in 18-27% cases and should be considered as a cause of renal artery stenosis in older patients with multisystem anomalies. Renal artery stenting in this subset can improve hypertension and renal outcomes. The disease has characteristic radiological findings and the diagnosis is frequently reliant on imaging. Histological examination is often not sufficient to confirm the diagnosis. ECD diagnosis is essential for treatment of the underlying disease process.