Childhood antineutrophil cytoplasmic antibodies associated vasculitides

The purpose of this review is to discuss the most recent published literature in childhood antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides and to identify the important issues arisen in the adult literature. This review will discuss the literature on new treatment regimens and outcome measurements.

Antineutrophil cytoplasmic antibodies are a hallmark of ANCA-associated vasculitides and are likely to be integral to the pathogenesis and have recently become a therapeutic target. In paediatrics, international collaborations have led to the development of childhood classification criteria, advanced understanding of the clinical phenotypes of childhood ANCA-associated vasculitides and improved our ability to capture disease severity, activity and damage. Treatment safety and efficacy data are mainly derived from adult studies and there is a lack of specific paediatric data. There are small case series on the efficacy of adult regimens in paediatric patients.

International multicentre studies are necessary for the evaluation of efficacy and safety of adult-derived treatment regimens in childhood ANCA-associated vasculitides. Specific childhood outcome measurements are recently developed for research purposes and enhance clinical care.