ANCA-associated vasculitis in childhood: recent advances

Ital J Pediatr. 2017 May 5;43(1):46. doi: 10.1186/s13052-017-0364-x.


Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in childhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of renal involvement and their prognostic implications, and outcome. Herein, we review the main clinical and therapeutic aspects of childhood-onset ANCA-associated vasculitis, and provide preliminary data on demographic characteristics and organ manifestations of an Italian multicentre cohort.

Keywords: ANCA; Autoimmunity; Childhood; Glomerulonephritis; Renal failure; Vasculitis.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Age Distribution
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / diagnosis*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / epidemiology*
  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Child
  • Child, Preschool
  • Churg-Strauss Syndrome / diagnosis
  • Churg-Strauss Syndrome / epidemiology
  • Female
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / epidemiology
  • Humans
  • Incidence
  • Male
  • Microscopic Polyangiitis / diagnosis
  • Microscopic Polyangiitis / epidemiology
  • Rare Diseases
  • Risk Assessment
  • Severity of Illness Index
  • Sex Distribution
  • Survival Rate


  • Antibodies, Antineutrophil Cytoplasmic