Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study

Background Pediatric ANCA vasculitis is a rare group of diseases with a scarcity of data in children. Annual incidence appeared to increase in the last several years, placing higher interest in the clinical and therapeutical outcomes of the disorder. Also, the growing use of rituximab questions the latest outcomes in these diseases. We therefore conducted a retrospective study to better understand the current characteristics, management, and the latest outcomes of the disorder. Methods We conducted a 9-year retrospective study of 46 children in 14 different centers across France to describe their clinical and laboratory presentations, therapeutic regimens, and kidney outcome. Results P-ANCA appeared to be a potential marker for higher relapse risk. Compared to adults, we found that ear-nose-throat presentations were frequent (45.7%) and more severe. Despite an evolution in the treatment management, kidney outcome remained poor with a substantial proportion of chronic kidney disease (54.8% at 1 year). Mortality stays low with 3 patients (6.5%) deceased at the end of our study. Conclusion Clinical presentation was as previously described and time to diagnosis remains long. P-ANCA is a statistically significant marker for increased relapse risk. We observed a modification in the treatment regimens over the past several years with a growing use of rituximab and a decreasing use of cyclophosphamide. Despite these changes, kidney outcome remains poor and prospective studies should be conducted to assess the most appropriate therapeutic modality for each patient. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information.

Medienart:

E-Artikel

Erscheinungsjahr:

2023

Erschienen:

2023

Enthalten in:

Zur Gesamtaufnahme - volume:38

Enthalten in:

Pediatric nephrology - 38(2023), 8 vom: 09. Jan., Seite 2649-2658

Sprache:

Englisch

Beteiligte Personen:

Mahi, Sarah-louisa [VerfasserIn]
Bahram, Siamak [VerfasserIn]
Harambat, Jérôme [VerfasserIn]
Allard, Lise [VerfasserIn]
Merlin, Etienne [VerfasserIn]
Belot, Alexandre [VerfasserIn]
Ranchin, Bruno [VerfasserIn]
Tenenbaum, Julie [VerfasserIn]
Magnavacca, Marie [VerfasserIn]
Haumesser, Lucile [VerfasserIn]
Allain-Launay, Emma [VerfasserIn]
Pietrement, Christine [VerfasserIn]
Flodrops, Hugues [VerfasserIn]
Ruin, Mahe [VerfasserIn]
Dossier, Claire [VerfasserIn]
Decramer, Stéphane [VerfasserIn]
Ballot-Schmitt, Claire [VerfasserIn]
Boyer, Olivia Gillion [VerfasserIn]
Seugé, Laure [VerfasserIn]
Ulinski, Tim [VerfasserIn]
Zaloszyc, Ariane [VerfasserIn]

Links:

Volltext [lizenzpflichtig]

Themen:

ANCA-associated vasculitis
Kidney outcome
Pediatric
Relapse

Anmerkungen:

© The Author(s), under exclusive licence to International Pediatric Nephrology Association 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

doi:

10.1007/s00467-022-05855-0

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

OLC2144793557

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