Medium Vessel Vasculitis

Kawasaki Disease

The peak age-of-onsetis at one year of age and 80% to 85% of cases occur at less than five years of age. Kawasaki disease (KD) is defined by the presence of fever persisting for at least five days, together with the presence of at least four of the following: changes of extremities (acute: erythema of palms, soles; oedema of hands, feet, and subacute: periungual peeling of fingers, toes in weeks 2 and 3); polymorphous exanthema; bilateral bulbar conjunctival injection without exudates; changes in lip and oral cavity: erythema, lips cracking, strawberry tongue, diffuse injection oral and pharyngeal mucosae; cervical lymphadenopathy (30). Initial treatment is with aspirin and intravenous immunoglobulin and further management is determined by the presence of coronary artery involvement, occurring in 20% to 30% of untreated cases. Although rare in adolescents, it does occur, and may be associated with more cardiovascular morbidity due to late presentation (30).

Polyarteritis Nodosa and Microscopic Polyangiitis

Polyarteritis nodosa (PAN) is observed in children and adolescents, but the peak age-of-onsetis around 50 years. Vasculitis of muscular medium sized vessels is characteristic, with aneurysm formation. Typically patients present with constitutional symptoms, abdominal pain, arthralgia, myalgia, livedo reticularis, peripheral neuropathy, hypertension and renal involvement (31). Angiography may demonstrate characteristic findings (32) but these are not specific to polyarteritis. Treatment is with corticosteroids, which are usually used in high dose to induce remission, and have reduced the five-year mortality from 90% to 50% (33). The addition of further immunosuppre-sion such as cyclophosphamide improves survival to 20% (34).

Microscopic polyarteritis (MPA) as defined in the CHCC may occur in children and adolescents (35) and may be differentiated from PAN with a clinical presentation of a severe renal-pulmonary syndrome. The characteristic pathologic lesion is necrotizing crescenteric glomerulonephritis without immunoglobulin or complement deposition. There is a strong association with ANCA and treatment is as for adults.

A postinfective vasculitis may present as a cutaneous polyarteritis and should be identified, as it carries a benign prognosis. The typical history is that of an upper respiratory tract infection followed by the appearance of a rash (typically tender nodules over the feet), myalgia, arthralgia/arthritis without internal organ involvement. Treatment is with NSAIDs, steroids and penicillin prophylaxis (34).

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