학술논문

Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study.
Document Type
Article
Source
Pediatric Nephrology. Aug2023, Vol. 38 Issue 8, p2649-2658. 10p. 1 Diagram, 3 Charts, 4 Graphs.
Subject
*CHRONIC kidney failure
*RITUXIMAB
*ANTINEUTROPHIL cytoplasmic antibodies
*RETROSPECTIVE studies
*ACQUISITION of data
*TREATMENT effectiveness
*DISEASE relapse
*SEVERITY of illness index
*MEDICAL records
*VASCULITIS
*OTOLARYNGOLOGY
*DISEASE risk factors
*SYMPTOMS
*CHILDREN
Language
ISSN
0931-041X
Abstract
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. [ABSTRACT FROM AUTHOR]