Pediatric Nephrology

, Volume 25, Issue 8, pp 1397–1407

ANCA-associated vasculitides—lessons from the adult literature

Authors

  • Joannis Vamvakopoulos
    • School of Immunity and Infection, College of Medical and Dental SciencesUniversity of Birmingham
  • Caroline O. Savage
    • School of Immunity and Infection, College of Medical and Dental SciencesUniversity of Birmingham
    • School of Immunity and Infection, College of Medical and Dental SciencesUniversity of Birmingham
Review

DOI: 10.1007/s00467-010-1496-z

Cite this article as:
Vamvakopoulos, J., Savage, C.O. & Harper, L. Pediatr Nephrol (2010) 25: 1397. doi:10.1007/s00467-010-1496-z

Abstract

Anti-neutrophil cytoplasmic antigen antibody (ANCA)-associated disease is a rare manifestation of primary systemic vasculitis in paediatric patients but one that carries significant morbidity, potential long-term disability and early mortality. It therefore requires a high index of suspicion, targeted investigation, prompt treatment and long-term follow-up with specialist input at every stage. The well-recognised diversity and overlap in clinical, laboratory and histopathological features of the ANCA-associated systemic vasculitides continue to hamper accurate diagnosis, confounding epidemiological data and necessitating a blanket approach to treatment, which is largely extrapolated from studies in adult patients and carries significant side-effects. Herein we summarise current knowledge of the epidemiology, pathogenesis, principal manifestations, investigation and evidence-based management, extrapolated from adult studies, of these disorders. We also discuss recent efforts towards classification of the childhood vasculitides that emphasise the value of histological diagnosis. Progress in our understanding of the pathophysiology underlying ANCA-associated disease should lead to targeted, safer and more effective therapies for these conditions. Nonetheless, many questions remain outstanding, and academic paediatricians face real challenges in identifying and collating the few cases they encounter into study cohorts. Meeting this challenge will require international collaboration, not only among paediatricians but also with the specialists taking over care of these patients as they reach adulthood.

Keywords

Microscopic polyangiitis/polyarteritisPaediatricVasculitisWegener’s granulomatosis

Abbreviations

ACR

American College of Rheumatology

ANCA

anti-neutrophil cytoplasmic antigen antibody

AASV

ANCA associated systemic vasculitis

CD

cluster designation

CHCC

Chapel Hill Consensus Conference

CPA

cyclophosphamide

CRP

C-reactive protein

CSS

Churg–Strauss syndrome

CT

computed tomography

CXR

chest x-ray

ESR

erythrocyte sedimentation rate

EUVAS

European Vasculitis Study Group

FcRγ

Fc receptor gamma chain

HLA

human leukocyte antigen

MPA

microscopic polyangiitis

MPO

myeloperoxidase

PR3

proteinase 3

RAG2

recombination activating gene 2

TNFα

tumour necrosis factor alpha

WG

Wegener’s granulomatosis

Copyright information

© IPNA 2010