Diagnosis and classification of Kawasaki disease.
Kawasaki disease is an acute systemic vasculitis of unknown etiology. Diagnosis is based on clinical criteria that include fever, exanthema, conjunctivitis, changes in the extremities, erythema of oral mucosa and lips and cervical lymphadenopathy. However, these criteria have low sensitivity and specificity and therefore, other clinical and laboratory features may be helpful in establishing the diagnosis, especially for cases of atypical or incomplete Kawasaki disease. Prognosis depends on the extent of cardiac involvement; coronary aneurysms develop in 20-25% of untreated patients and these may lead to myocardial infarction and sudden death. Treatment with high-dose intravenous immunoglobulin is effective in reducing the risk of coronary aneurysms in most cases and is the treatment of choice for initial Kawasaki disease.
Pediatric Rheumatology Unit, Hospital Sant Joan de Déu, Universitat de Barcelona, Spain. Electronic address: jsanchezm@hsjdbcn.org.
Judith
J
Sánchez-Manubens
Pediatric Rheumatology Unit, Hospital Sant Joan de Déu, Universitat de Barcelona, Spain. Electronic address: rbou@hsjdbcn.org.
Rosa
R
Bou
Pediatric Rheumatology Unit, Hospital Sant Joan de Déu, Universitat de Barcelona, Spain. Electronic address: janton@hsjdbcn.org.
Jordi
J
Anton
D000208:Acute Disease; D001208:Asia / Q000453:epidemiology; D001323:Autoantibodies / Q000096:biosynthesis; D002140:California / Q000453:epidemiology; D020022:Genetic Predisposition to Disease; D055106:Genome-Wide Association Study; D006254:Hawaii / Q000453:epidemiology; D006801:Humans; D007070:Immunoglobulin A / Q000096:biosynthesis; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D015994:Incidence; D009080:Mucocutaneous Lymph Node Syndrome / Q000145:classification* / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics
D001323:Autoantibodies; D007070:Immunoglobulin A; D016756:Immunoglobulins, Intravenous
Child; Coronary aneurysm; Fever; Intravenous immunoglobulins; Mucocutaneous lymph node syndrome