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

dc.rights.licenseopenen_US
dc.contributor.authorMAHI, Sarah-Louisa
dc.contributor.authorBAHRAM, Siamak
hal.structure.identifierBordeaux population health [BPH]
dc.contributor.authorHARAMBAT, Jerome
dc.contributor.authorALLARD, Lise
dc.contributor.authorMERLIN, Etienne
dc.contributor.authorBELOT, Alexandre
dc.contributor.authorRANCHIN, Bruno
dc.contributor.authorTENENBAUM, Julie
dc.contributor.authorMAGNAVACCA, Marie
dc.contributor.authorHAUMESSER, Lucile
dc.contributor.authorALLAIN-LAUNAY, Emma
dc.contributor.authorPIETREMENT, Christine
dc.contributor.authorFLODROPS, Hugues
dc.contributor.authorRUIN, Mahe
dc.contributor.authorDOSSIER, Claire
dc.contributor.authorDECRAMER, Stephane
dc.contributor.authorBALLOT-SCHMITT, Claire
dc.contributor.authorBOYER, Olivia Gillion
dc.contributor.authorSEUGE, Laure
dc.contributor.authorULINSKI, Tim
dc.contributor.authorZALOSZYC, Ariane
dc.date.accessioned2023-02-22T14:32:09Z
dc.date.available2023-02-22T14:32:09Z
dc.date.issued2023-01-09
dc.identifier.issn1432-198X (Electronic) 0931-041X (Linking)en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/172061
dc.description.abstractEnBACKGROUND: 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.
dc.language.isoENen_US
dc.subject.enANCA-associated vasculitis
dc.subject.enPediatric
dc.subject.enRelapse
dc.subject.enKidney outcome
dc.title.enPediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study
dc.title.alternativePediatr Nephrolen_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1007/s00467-022-05855-0en_US
dc.subject.halSciences du Vivant [q-bio]/Santé publique et épidémiologieen_US
dc.identifier.pubmed36622443en_US
bordeaux.journalPediatric Nephrologyen_US
bordeaux.hal.laboratoriesBordeaux Population Health Research Center (BPH) - UMR 1219en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.institutionINSERMen_US
bordeaux.teamLEHA_BPHen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
hal.identifierhal-04000905
hal.version1
hal.date.transferred2023-02-22T14:32:21Z
hal.exporttrue
dc.rights.ccPas de Licence CCen_US
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