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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

Authors

  • 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
Background

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).

Methods

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.

Results

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).

Conclusion

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.

Funding

  • Commercial Support