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Pediatric Rheumatology
Published in: Pediatric Rheumatology 1/2019

Open Access 01-12-2019 | ANCA-Associated Vasculitis | Review

Hallmark trials in ANCA-associated vasculitis (AAV) for the pediatric rheumatologist

Authors: Jennifer J. Y. Lee, Alhanouf Alsaleem, Grace P. K. Chiang, Elizaveta Limenis, Watchareewan Sontichai, Rae S. M. Yeung, Jonathan Akikusa, Ronald M. Laxer

Published in: Pediatric Rheumatology | Issue 1/2019

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Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a complex group of systemic vasculitides that are characterized by primary small-to-medium sized blood vessel inflammation with the presence of autoantibodies known as ANCA. AAV diseases include Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA), and Microscopic Polyangiitis (MPA). AAVs are challenging conditions associated with high cumulative disease and treatment related morbidity and mortality. Given its rarity and the resulting paucity of pediatric-specific clinical trial evidence, pediatric rheumatologists have had to often extrapolate from adult literature for management and therapeutic decisions. The aim of this review is to provide a comprehensive overview of the important findings and overall conclusions of critical landmark clinical trials in the induction and maintenance treatments in adult AAV for the pediatric rheumatologist. This review also highlights the outcomes of recent pediatric AAV observational studies and discusses the future research priorities in pediatric AAV management.
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Metadata
Title
Hallmark trials in ANCA-associated vasculitis (AAV) for the pediatric rheumatologist
Authors
Jennifer J. Y. Lee
Alhanouf Alsaleem
Grace P. K. Chiang
Elizaveta Limenis
Watchareewan Sontichai
Rae S. M. Yeung
Jonathan Akikusa
Ronald M. Laxer
Publication date
01-12-2019

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