Pediatric Vasculitis

• Kawasaki disease (KD) typically occurs in children under the age of 5 and has a striking predilection for Asian children. • The diagnosis of KD should be considered in any child with prolonged fever and neck swelling that does not respond to antibiotics. • Early intervention with IVIG prevents some cases of coronary artery aneurysms in KD—but not all. • IgA vasculitis is a small-vessel vasculitis typified by palpable purpura but also associated with joint, gastrointestinal, and renal involvement in many patients. • Polyarteritis nodosa is a medium-vessel vasculitis often considered to have both cutaneous and systemic forms. These subsets are likely different diseases altogether, with contrasting treatment considerations. • Monogenic vasculitides associated with autoinflammatory syndromes often mimic polyarteritis closely but respond well to different therapeutic approaches. Biologic agents such as those focusing on tumor necrosis factor inhibition are often highly effective. • Granulomatosis with polyangiitis in children behaves similarly to disease in adults.