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15-06-2017 | Vasculitis | Review | Article

Renal Vasculitis in Childhood

Journal:
Current Pediatrics Reports

Authors: Sally Kellett, Natasha Jawa, Damien Noone

Publisher: Springer US

Abstract

Renal vasculitis in children, while rare, can be life threatening and associated with significant morbidity and mortality. Although sometimes renal limited, more often, the kidney is affected as part of a more systemic disorder. While there are increasing numbers of pediatric case series, management decisions are derived from randomized controlled trials conducted in adults. The aim of this review is to summarize the recent literature on the pathogenesis, management, and outcome of small-, medium-, and large-vessel renal vasculitis in the pediatric population.
Our understanding of the pathogenesis of these conditions has grown allowing more targeted therapies, such as rituximab or potentially complement pathway inhibitors in ANCA vasculitis and biologicals in polyarteritis nodosa and Takayasu’s arteritis. There is a new and validated histopathological classification schema that predicts renal outcome in pediatric ANCA vasculitis.
The results of key adult studies on optimal duration of immunosuppression maintenance are keenly awaited.

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