Atheroembolic Kidney Disease and Atypical Hemolytic Uremic Syndrome: Two Sides of the Same Coin?

Introduction: Atheroembolic kidney disease (AEKD) is an under-recognized cause of kidney failure, secondary to the obstruction of the renal artery and/or its branches due to the rupture of an unstable atherosclerotic plaque in patients treated with surgical and invasive cardiovascular pro...

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Main Authors: Antonio Pisani, Pasquale Buonanno, Maria Amicone, Eleonora Riccio, Ivana Capuano
Format: Article
Language:English
Published: Karger Publishers 2025-01-01
Series:Kidney & Blood Pressure Research
Online Access:https://karger.com/article/doi/10.1159/000542788
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author Antonio Pisani
Pasquale Buonanno
Maria Amicone
Eleonora Riccio
Ivana Capuano
author_facet Antonio Pisani
Pasquale Buonanno
Maria Amicone
Eleonora Riccio
Ivana Capuano
author_sort Antonio Pisani
collection DOAJ
description Introduction: Atheroembolic kidney disease (AEKD) is an under-recognized cause of kidney failure, secondary to the obstruction of the renal artery and/or its branches due to the rupture of an unstable atherosclerotic plaque in patients treated with surgical and invasive cardiovascular procedures. The embolization of cholesterol crystals in the renal artery activates the complement and triggers an inflammatory reaction. Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy caused by the hyperactivation of the alternative complement pathway, leading to a prothrombotic and proinflammatory state on the endothelial surface. AEKD and aHUS could share the involvement of the complement in their pathophysiological mechanism and the former could lead to the latter. Case Presentation: A 72-year-old man was referred to our clinic because of a rapid worsening of renal function after 9 months from an endovascular aortic repair (EVAR). After 4 months from the intervention, his renal function worsened, he developed hypereosinophilia and skin lesions; the renal ultrasound showed increased resistance indexes, strongly suggestive of atheroembolic kidney disease. Successively, we observed thrombocytopenia, anemia, increased LDH, low plasmatic haptoglobin, schistocytes in blood smear, and normal ADAMTS13. We promptly diagnosed an atypical hemolytic uremic syndrome and started ravulizumab. Conclusion: To our knowledge, this is the first case of aHUS secondary to a subacute AEKD. Further studies are necessary to fill the gap in the knowledge of the precise mechanism leading to aHUS secondary to AEKD and to confirm that they are two sides of the same coin.
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spelling doaj-art-152ffca9e3bb4fce8deb275324b648b72025-08-20T03:09:47ZengKarger PublishersKidney & Blood Pressure Research1423-01432025-01-0150117117510.1159/000542788Atheroembolic Kidney Disease and Atypical Hemolytic Uremic Syndrome: Two Sides of the Same Coin?Antonio PisaniPasquale BuonannoMaria AmiconeEleonora RiccioIvana Capuano Introduction: Atheroembolic kidney disease (AEKD) is an under-recognized cause of kidney failure, secondary to the obstruction of the renal artery and/or its branches due to the rupture of an unstable atherosclerotic plaque in patients treated with surgical and invasive cardiovascular procedures. The embolization of cholesterol crystals in the renal artery activates the complement and triggers an inflammatory reaction. Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy caused by the hyperactivation of the alternative complement pathway, leading to a prothrombotic and proinflammatory state on the endothelial surface. AEKD and aHUS could share the involvement of the complement in their pathophysiological mechanism and the former could lead to the latter. Case Presentation: A 72-year-old man was referred to our clinic because of a rapid worsening of renal function after 9 months from an endovascular aortic repair (EVAR). After 4 months from the intervention, his renal function worsened, he developed hypereosinophilia and skin lesions; the renal ultrasound showed increased resistance indexes, strongly suggestive of atheroembolic kidney disease. Successively, we observed thrombocytopenia, anemia, increased LDH, low plasmatic haptoglobin, schistocytes in blood smear, and normal ADAMTS13. We promptly diagnosed an atypical hemolytic uremic syndrome and started ravulizumab. Conclusion: To our knowledge, this is the first case of aHUS secondary to a subacute AEKD. Further studies are necessary to fill the gap in the knowledge of the precise mechanism leading to aHUS secondary to AEKD and to confirm that they are two sides of the same coin. https://karger.com/article/doi/10.1159/000542788
spellingShingle Antonio Pisani
Pasquale Buonanno
Maria Amicone
Eleonora Riccio
Ivana Capuano
Atheroembolic Kidney Disease and Atypical Hemolytic Uremic Syndrome: Two Sides of the Same Coin?
Kidney & Blood Pressure Research
title Atheroembolic Kidney Disease and Atypical Hemolytic Uremic Syndrome: Two Sides of the Same Coin?
title_full Atheroembolic Kidney Disease and Atypical Hemolytic Uremic Syndrome: Two Sides of the Same Coin?
title_fullStr Atheroembolic Kidney Disease and Atypical Hemolytic Uremic Syndrome: Two Sides of the Same Coin?
title_full_unstemmed Atheroembolic Kidney Disease and Atypical Hemolytic Uremic Syndrome: Two Sides of the Same Coin?
title_short Atheroembolic Kidney Disease and Atypical Hemolytic Uremic Syndrome: Two Sides of the Same Coin?
title_sort atheroembolic kidney disease and atypical hemolytic uremic syndrome two sides of the same coin
url https://karger.com/article/doi/10.1159/000542788
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