GENERAL OBJECTIVE

• Determine the clinical features and outcomes of childhood-onset anti-

neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV),
particularly microscopic polyangiitis (MPA)
Introduction
• Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a
group of disorders characterized by necrotizing inflammation of small- to
medium-sized vessels
• GPA (Wegener’s Granulomatosis)
• MPA
• EGPA (Churg-Strauss Syndrome)

• AAV generally occurs in adults at 5th-7th decades of life

•Epidemiologic data, Incidence, Prevalence and Disease course of AAV in

pediatric patients are scarce and poorly understood
Introduction
• This nationwide study was conducted to investigate the principal
clinical and demographic features of pediatric AAV at presentation,
disease course, and identify factors associated with progression to
ESRD