Enfermedad de Kawasaki y vasculitis de pequeño vaso

Abstract

Kawasaki disease is a vasculitis that affects medium-sized vessels, especially the coronary arteries and children under 5 years of age, and is accompanied by mucocutaneous lesions and adenopathy. There are established criteria for classifying the disease. Treatment should be early and is indicated when these criteria are met based on the combination of immunoglobulins and acetylsalicylic acid. Small vessel vasculitis, according to the International Chapel Hill Consensus Conference includes ANCA-associated vasculitis and immune complex deposition vasculitis. The latter group includes IgA vasculitis, cryoglobulinaemic vasculitis, hypocomplementaemic urticarial vasculitis and anti-glomerular basement membrane antibody vasculitis. Diagnosis in most cases requires evidence of small vessel vasculitis on biopsy, but for anti-glomerular basement membrane antibody vasculitis this is not essential. The severity of the manifestations of each will determine treatment. Anti-glomerular basement membrane vasculitis requires intensive treatment from onset based on cyclophosphamide, corticosteroids, and plasma exchange. In all the other entities the treatment regimen depends on the severity patterns. The prognosis is also different, IgA vasculitis and urticarial vasculitis tend to have a better prognosis, and cryoglobulinaemic vasculitis and especially anti- glomerular basement membrane antibody vasculitis have a worse prognosis.