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: | , , , , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
August 2024
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| 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 |
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| 245 | 1 | 0 | |a Characterization of patients with aHUS and associated triggers or clinical conditions |b a Global aHUS Registry analysis |c 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 |
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| 520 | |a 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. | ||
| 650 | 4 | |a aHUS | |
| 650 | 4 | |a anti-CFH | |
| 650 | 4 | |a associated condition | |
| 650 | 4 | |a complement | |
| 650 | 4 | |a genetic | |
| 650 | 4 | |a trigger | |
| 700 | 1 | |a Al-Dakkak, Imad |e VerfasserIn |4 aut | |
| 700 | 1 | |a Anokhina, Katerina |e VerfasserIn |4 aut | |
| 700 | 1 | |a Isbel, Nicole |e VerfasserIn |4 aut | |
| 700 | 1 | |a Frémeaux-Bacchi, Véronique |e VerfasserIn |4 aut | |
| 700 | 1 | |a Gilbert, Rodney D. |e VerfasserIn |4 aut | |
| 700 | 1 | |a Greenbaum, Larry A. |e VerfasserIn |4 aut | |
| 700 | 1 | |a Ariceta, Gema |e VerfasserIn |4 aut | |
| 700 | 1 | |a Ardissino, Gianluigi |e VerfasserIn |4 aut | |
| 700 | 1 | |a Schaefer, Franz |d 1961- |e VerfasserIn |0 (DE-588)1023365383 |0 (DE-627)718365577 |0 (DE-576)366705598 |4 aut | |
| 700 | 1 | |a Rondeau, Eric |e VerfasserIn |4 aut | |
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