Renal Vasculitis

The nomenclature of vasculitides has been revised in light of recent understanding of the pathogenesis of these diseases. The Ankara 2008 childhood classification criteria aid in the management of pediatric patients with vasculitis. The most common childhood vasculitis, IgA vasculitis (Henoch-Schönlein purpura) is reviewed elsewhere. The other common vasculitis of childhood, Kawasaki disease affects medium-sized arteries but rarely the kidney. ANCA-associated vasculitis (AAV) often affects the kidney, mainly in form of pauci-immune necrotizing glomerulonephritis. The most common AAV is granulomatosis with polyangiitis (Wegener’s granulomatosis). Polyarteritis nodosa (PAN) is a medium-sized arteritis that may involve the arteries of the kidneys as well. Deficiency of adenosine deaminase (ADA2) might mimic PAN and should be considered in patients with features of PAN with family history, or those with stroke or with hematological and/or immunological abnormalities. Takayasu arteritis and rarely Behçet disease can also affect the vessels of the kidney. Recent management and treatment strategies guide us in the care of these children.