ANCA-associated vasculitis in childhood: recent advances

M. Calatroni, E. Oliva, D. Gianfreda, G. Gregorini, M. Allinovi, G. A. Ramirez, E. P. Bozzolo, S. Monti, C. Bracaglia, G. Marucci, M. Bodria, R. A. Sinico, F. Pieruzzi, G. Moroni, S. Pastore, G. Emmi, P. Esposito, M. Catanoso, G. Barbano, A. BonanniA. Vaglio

Research output: Contribution to journalArticle

Abstract

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.
Original languageEnglish
Pages (from-to)46-017-0364-x
JournalItalian Journal of Pediatrics
Volume43
Issue number1
DOIs
Publication statusPublished - May 5 2017

Keywords

  • ANCA
  • Autoimmunity
  • Childhood
  • Glomerulonephritis
  • Renal failure
  • Vasculitis

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    Calatroni, M., Oliva, E., Gianfreda, D., Gregorini, G., Allinovi, M., Ramirez, G. A., Bozzolo, E. P., Monti, S., Bracaglia, C., Marucci, G., Bodria, M., Sinico, R. A., Pieruzzi, F., Moroni, G., Pastore, S., Emmi, G., Esposito, P., Catanoso, M., Barbano, G., ... Vaglio, A. (2017). ANCA-associated vasculitis in childhood: recent advances. Italian Journal of Pediatrics, 43(1), 46-017-0364-x. https://doi.org/10.1186/s13052-017-0364-x [doi]