Pediatric Nephrology

, Volume 25, Issue 9, pp 1641–1652

Medium-size-vessel vasculitis

  • Michael J. Dillon
  • Despina Eleftheriou
  • Paul A. Brogan
Educational Review

Abstract

Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis.

Keywords

Vasculitis Polyarteritis nodosa Cutaneous polyarteritis nodosa Kawasaki disease Child 

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Copyright information

© IPNA 2009

Authors and Affiliations

  • Michael J. Dillon
    • 1
  • Despina Eleftheriou
    • 2
  • Paul A. Brogan
    • 2
  1. 1.Nephrourology UnitUCL Institute of Child HealthLondonUK
  2. 2.Department of RheumatologyUCL Institute of Child HealthLondonUK

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