Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors

Détails

ID Serval
serval:BIB_4A7C9A825EB6
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors
Périodique
Kidney Int
Auteur⸱e⸱s
Le Clech A., Simon-Tillaux N., Provot F., Delmas Y., Vieira-Martins P., Limou S., Halimi J. M., Le Quintrec M., Lebourg L., Grange S., Karras A., Ribes D., Jourde-Chiche N., Rondeau E., Fremeaux-Bacchi V., Fakhouri F.
ISSN
1523-1755 (Electronic)
ISSN-L
0085-2538
Statut éditorial
Publié
Date de publication
06/2019
Volume
95
Numéro
6
Pages
1443-1452
Langue
anglais
Notes
Le Clech, Alice
Simon-Tillaux, Noemie
Provot, Francois
Delmas, Yahsou
Vieira-Martins, Paula
Limou, Sophie
Halimi, Jean-Michel
Le Quintrec, Moglie
Lebourg, Ludivine
Grange, Steven
Karras, Alexandre
Ribes, David
Jourde-Chiche, Noemie
Rondeau, Eric
Fremeaux-Bacchi, Veronique
Fakhouri, Fadi
eng
Comparative Study
Research Support, Non-U.S. Gov't
Kidney Int. 2019 Jun;95(6):1443-1452. doi: 10.1016/j.kint.2019.01.023. Epub 2019 Mar 15.
Résumé
Secondary hemolytic uremic syndrome (HUS) is a heterogeneous group of thrombotic microangiopathies associated with various underlying conditions. Whether it belongs to the spectrum of complement-mediated HUS remains controversial. We analysed the presentation, outcome, and frequency of complement gene rare variants in a cohort of 110 patients with secondary HUS attributed to drugs (29%), autoimmune diseases (24%), infections (17%), malignancies (10%), glomerulopathies (9%), extra-renal organ transplantation (8%), and pancreatitis (3%). The frequency of complement gene rare variants was similar in patients with secondary HUS (5%) and in healthy individuals (6% and 8% in French and European controls, respectively). At diagnosis, 40% of patients required dialysis and 18% had neurological manifestations. Fifty percent of patients received plasmatherapy and 35% were treated with eculizumab. Haematological and complete renal remission was achieved in 80% and 24% of patients, respectively. Thirty-nine percent of patients progressed to chronic kidney disease (stages 3-4) and an additional 37% reached end-stage renal disease. Eleven percent of patients died, most often from complications of the underlying cause of HUS. Only one patient experienced an HUS relapse. Patients treated with eculizumab presented with more severe HUS and were more likely to require dialysis at the time of diagnosis as compared to patients not treated with eculizumab. Rates of hematological remission, chronic kidney disease (stages 3-4), and end-stage renal disease were similar in the two groups. Secondary HUS is an acute nonrelapsing form of HUS, not related to complement dysregulation. The efficacy of eculizumab in this setting is not yet established.
Mots-clé
Adolescent, Adult, Aged, Aged, 80 and over, Antibodies, Monoclonal, Humanized/therapeutic use, Atypical Hemolytic Uremic Syndrome/*genetics/mortality/pathology/therapy, Child, Child, Preschool, Complement Activation/genetics, Complement Inactivating Agents/therapeutic use, Complement System Proteins/*genetics/immunology, Disease Progression, Female, France/epidemiology, Hemolytic-Uremic Syndrome/*etiology/mortality/pathology/therapy, Humans, Kidney/immunology/pathology, Kidney Failure, Chronic/*epidemiology/pathology, Male, Middle Aged, Plasmapheresis/statistics & numerical data, Registries/statistics & numerical data, Renal Dialysis/statistics & numerical data, Renal Insufficiency, Chronic/*epidemiology/pathology, Retrospective Studies, Risk Factors, Treatment Outcome, Young Adult, *complement, *eculizumab, *hemolytic uremic syndrome, *thrombotic microangiopathy
Pubmed
Création de la notice
01/03/2022 11:17
Dernière modification de la notice
02/03/2022 7:35
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