Primary Vasculitis in Childhood: GPA and MPA in Childhood

Frontiers in Pediatrics
Mehul P Jariwala, Ronald M Laxer

Abstract

Childhood onset anti-neutrophilic cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a rare group of primary systemic vasculitides affecting medium and small blood vessels. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and renal limited ANCA vasculitis. These disorders are associated with severe clinical manifestations, frequent relapses and a high cumulative morbidity, and often present with multisystem involvement. Renal involvement is common in the pediatric age group, characterized by pauci-immune necrotizing and crescentic glomerulonephritis which frequently progresses to chronic kidney disease in adulthood. ANCAs against proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO) (MPO-ANCA) remain the hallmark of AAV and are integral to the disease pathogenesis. Newer understanding of neutrophil extracellular traps and complement activation have provided better insights into disease pathogenesis. A pediatric vasculitis working group has developed and validated childhood vasculitis classification criteria and disease activity and damage scores. No specific pediatric treatment recommendations exist due to rare nature of the illness in ...Continue Reading

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Citations

Aug 31, 2019·Current Rheumatology Reports·Renato Ferrandiz-Espadin, Manuel Ferrandiz-Zavaler
Jul 29, 2020·Frontiers in Pediatrics·Martin SmitkaMaja von der Hagen
Dec 8, 2019·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Luisa Safar-BoueriRivka Ayalon
Jun 2, 2020·Frontiers in Pediatrics·Antonio MastrangeloGiovanni Montini
Jan 30, 2021·Current Rheumatology Reports·Mehul P Jariwala, Ronald M Laxer
Mar 9, 2021·Respirology Case Reports·Matthew D Wong, Leanne M Gauld
May 1, 2021·European Respiratory Review : an Official Journal of the European Respiratory Society·Sebastian F N BodeAndrea Heinzmann
Jun 7, 2021·Pediatric Rheumatology Online Journal·Rotem Semo OzLinda Wagner-Weiner
Jun 29, 2021·Radiology Case Reports·Sekhar IyerLyle Gesner
Oct 30, 2020·Pediatrics·Timothy G SingerCarla Falco

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Methods Mentioned

BETA
ELISA
biopsy
ESR

Clinical Trials Mentioned

NCT01731561
NCT01663623
NCT02108860
NCT01363388
NCT02994927
NCT01446211

Software Mentioned

CanVasc
SPARROW
BVAS

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