Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study - Archive ouverte HAL
Article Dans Une Revue Pediatric Nephrology Année : 2023

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

Sarah-Louisa Mahi
  • Fonction : Auteur
Siamak Bahram
  • Fonction : Auteur
Lise Allard
  • Fonction : Auteur
Etienne Merlin
  • Fonction : Auteur
Alexandre Belot
Bruno Ranchin
  • Fonction : Auteur
Julie Tenenbaum
  • Fonction : Auteur
Marie Magnavacca
  • Fonction : Auteur
Lucile Haumesser
  • Fonction : Auteur
Emma Allain-Launay
  • Fonction : Auteur
Christine Pietrement
  • Fonction : Auteur
Hugues Flodrops
  • Fonction : Auteur
Mahe Ruin
  • Fonction : Auteur
Claire Dossier
  • Fonction : Auteur
Stephane Decramer
  • Fonction : Auteur
Claire Ballot-Schmitt
  • Fonction : Auteur
Olivia Gillion Boyer
  • Fonction : Auteur
Laure Seuge
  • Fonction : Auteur
Tim Ulinski
  • Fonction : Auteur
Ariane Zaloszyc
  • Fonction : Auteur

Résumé

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. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Dates et versions

hal-04000905 , version 1 (22-02-2023)

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Sarah-Louisa Mahi, Siamak Bahram, Jerome Harambat, Lise Allard, Etienne Merlin, et al.. Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study. Pediatric Nephrology, 2023, ⟨10.1007/s00467-022-05855-0⟩. ⟨hal-04000905⟩

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