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Abstract: TH-PO500

Characterization of Patients With Thrombotic Microangiopathy and Triggering/Associated Events: A Global aHUS Registry Analysis

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials


  • Siedlecki, Andrew M., Department of Internal Medicine, Renal Division, Brigham and Women's Hospital, Boston, Massachusetts, United States
  • Al-Dakkak, Imad, Alexion, AstraZeneca Rare Disease, Boston, Massachusetts, United States
  • Anokhina, Ekaterina, Alexion, AstraZeneca Rare Disease, Boston, Massachusetts, United States
  • Isbel, Nicole, Department of Nephrology, Princess Alexandra Hospital, Brisbane, Queensland, Australia
  • Fremeaux-Bacchi, Veronique, Laboratoire d'Immunologie, Hôpital Européen Georges Pompidou, Paris, France
  • Gilbert, Rodney David, Regional Paediatric Nephro-Urology Unit, Southampton Children's Hospital, Tremona Road, Southampton, United Kingdom
  • Greenbaum, Larry A., Division of Pediatric Nephrology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, Georgia, United States
  • Ariceta, Gema, Department of Pediatric Nephrology, Vall d'Hebron Hospital, and the Autonomous University of Barcelona, Barcelona, Spain
  • Ardissino, Gianluigi, Centro per la Cura e lo Studio della Sindrome Emolitico-Uremica, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
  • Schaefer, Franz S., Division of Pediatric Nephrology, Heidelberg University Hospital, Heidelberg, Germany
  • Rondeau, Eric, Urgences Néphrologiques et Transplantation Rénale, Hôpital Tenon, Paris, France
  • Licht, Christoph, Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada

Atypical hemolytic uremic syndrome (aHUS), a rare disease of complement dysregulation, leads to thrombotic microangiopathy (TMA). In this study, we characterize patients (pts) with triggering/associated events occurring prior/up to aHUS onset from the Global aHUS Registry (NCT01522183).


All pts with triggering/associated events prior/up to aHUS onset and enrolled in the Registry from 2011 to July 2021 were included. Pts with an alternative clinical diagnosis after enrollment were excluded.


In total, 349 pts with triggering/associated events were included, of which 75% were adults (≥ 18 years) (n=229) and 25% (n=78) were pediatric pts (<18 years). Most pts (n=307, 88%) had only one triggering/associated event, and in those, the most frequent triggering/associated events were malignancy in adults (n=51; 22%) and acute infection (n=28; 36%) in pediatric pts. The mean time from event to aHUS onset in these categories were 22 and 0.03 months, respectively. The least frequent were bone marrow transplant (1.7%) and C3 glomerulopathy (0.9%) in adults, and C3 glomerulopathy (0%), and drug-induced aHUS (0%) in pediatric pts. Autoimmune disorders (n=36) had the longest time from event to aHUS onset (41 months), while acute infection (n=53) and malignant hypertension (n=26) had the shortest (0.03 months). The most common triggering and associated events combinations were drug-induced aHUS and malignancy (n=7).


This study suggests that triggering events can be organized into clinically relevant categories. The frequency of triggering/associated events occurring prior/up to aHUS differ in pediatric and adult populations. The interval between triggering/associated events and the eventual diagnosis of aHUS differ by type of event.


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