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Characterization of patients with aHUS and associated triggers or clinical conditions: a Global aHUS Registry analysis

Introduction Atypical haemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) associated with complement dysregulation; aHUS may be associated with other ‘triggers’ or ‘clinical conditions’. This study aimed to characterize this patient population using data from the Glo...

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Hauptverfasser: Licht, Christoph (VerfasserIn) , Al-Dakkak, Imad (VerfasserIn) , Anokhina, Katerina (VerfasserIn) , Isbel, Nicole (VerfasserIn) , Frémeaux-Bacchi, Véronique (VerfasserIn) , Gilbert, Rodney D. (VerfasserIn) , Greenbaum, Larry A. (VerfasserIn) , Ariceta, Gema (VerfasserIn) , Ardissino, Gianluigi (VerfasserIn) , Schaefer, Franz (VerfasserIn) , Rondeau, Eric (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: August 2024
In: Nephrology
Year: 2024, Jahrgang: 29, Heft: 8, Pages: 519-527
ISSN:1440-1797
DOI:10.1111/nep.14304
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1111/nep.14304
Verlag, lizenzpflichtig, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1111/nep.14304
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Verfasserangaben:Christoph Licht, Imad Al-Dakkak, Katerina Anokhina, Nicole Isbel, Véronique Frémeaux-Bacchi, Rodney D. Gilbert, Larry A. Greenbaum, Gema Ariceta, Gianluigi Ardissino, Franz Schaefer, Eric Rondeau
Beschreibung
Zusammenfassung:Introduction Atypical haemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) associated with complement dysregulation; aHUS may be associated with other ‘triggers’ or ‘clinical conditions’. This study aimed to characterize this patient population using data from the Global aHUS Registry, the largest collection of real-world data on patients with aHUS. Methods Patients enrolled in the Global aHUS Registry between April 2012 and June 2021 and with recorded aHUS-associated triggers or clinical conditions prior/up to aHUS onset were analysed. aHUS was diagnosed by the treating physician. Data were classified by age at onset of aHUS (< or ≥18 years) and additionally by the presence/absence of identified pathogenic complement genetic variant(s) and/or anti-complement factor H (CFH) antibodies. Genetically/immunologically untested patients were excluded. Results 1947 patients were enrolled in the Global aHUS Registry by June 2021, and 349 (17.9%) met inclusion criteria. 307/349 patients (88.0%) had a single associated trigger or clinical condition and were included in the primary analysis. Malignancy was most common (58/307, 18.9%), followed by pregnancy and acute infections (both 53/307, 17.3%). Patients with an associated trigger or clinical condition were generally more likely to be adults at aHUS onset. Conclusion Our analysis suggests that aHUS-associated triggers or clinical conditions may be organized into clinically relevant categories, and their presence does not exclude the concurrent presence of pathogenic complement genetic variants and/or anti-CFH antibodies. Considering a diagnosis of aHUS with associated triggers or clinical conditions in patients presenting with TMA may allow faster and more appropriate treatment.
Beschreibung:Veröffentlicht: 11 April 2024
Gesehen am 02.10.2024
Beschreibung:Online Resource
ISSN:1440-1797
DOI:10.1111/nep.14304

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