Diagnosis and classification of Kawasaki disease

• Published in: Journal of autoimmunity 2014-02, Vol.48, p.113-117
• Main Authors: Sánchez-Manubens, Judith, Bou, Rosa, Anton, Jordi
• Format: Article
• Language: English
• Subjects: Acute Disease; Allergy and Immunology; Asia - epidemiology; Autoantibodies - biosynthesis; Biological and medical sciences; Blood and lymphatic vessels; California - epidemiology; Cardiology. Vascular system; Child; Coronary aneurysm; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Fever; Fundamental and applied biological sciences. Psychology; Fundamental immunology; Genetic Predisposition to Disease; Genome-Wide Association Study; Hawaii - epidemiology; Humans; Immunoglobulin A - biosynthesis; Immunoglobulins, Intravenous - therapeutic use; Incidence; Intravenous immunoglobulins; Medical sciences; Mucocutaneous lymph node syndrome; Mucocutaneous Lymph Node Syndrome - classification; Mucocutaneous Lymph Node Syndrome - diagnosis; Mucocutaneous Lymph Node Syndrome - epidemiology; Mucocutaneous Lymph Node Syndrome - genetics; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
• ISSN: 0896-8411; 1095-9157
• DOI: 10.1016/j.jaut.2014.01.010

Abstract 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.