Abordaje diagnóstico y terapéutico de la enfermedad de Kawasaki

Kawasaki disease (KD) is an acute vasculitis, of unknown etiology of very young children. Its main complication is the formation of coronary artery aneurysms. The diagnosis is eminently clinical based on the presence of fever for a minimum of five days plus four of other five main criteria i.e., 1) erythema, lip fissures, strawberry tongue, pharyngeal hyperemia; 2) conjunctive hyperemia; 3) maculopapular exanthema; 4) extremities erythema, edema, palmar, plantar perianal exfoliation; 5) cervical adenopathy. The diagnosis of atypical or incomplete cases of KD is based on the presence of fever and less than the four criteria required, associated with coronary involvement detected by echocardiography or coronary angiography. Treatment with intravenous gammaglobulina has reduced the prevalence of coronary dilation to less than 5% and of giant aneurysms to less than 1%, while mortality has decreased from 2% to 0.3%. However, some patients who were treated early on develop heart involvement. The purpose of this review is to sensitize the medical profession on this pathology; also to give a detailed description of the diagnostic approaches, and to illustrate most of the clinical features of the disease. Additional manifestations not included in the main approaches are useful to establish an early diagnosis either of classic or of incomplete Kawasaki disease. We discuss the ideal timing to begin treatment, and the indication for the use of intravenous gammaglobulin ten days after the appearance of fever and which patients require larger and/or additional doses of gammaglobulin; to determine which patients require steroids. Keyword: Kawasaki disease, incomplete Kawasaki, coronary aneurysm, intravenous gammaglobulin Articulo de revision