1Faculty of Medicine, Vilnius university
Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome and Kawasaki syndrome, is an inflammatory disease in children associated with vasculitis affecting predominantly the coronary arteries and is now the most common cause of acquired heart disease in children in developed countries. The etiology of KD is unknown but epidemiological studies implicate an infectious agent or toxin, which causes disease in genetically predisposed individuals. Clinical manifestation remains the most important tool for diagnosis. Clinical features include fever for more than 5 days, rash, conjunctivitis, oral mucositis, extremity changes, cervical lymphadenopathy, and, in a proportion of cases, dilation or aneurysms of the coronary and other arteries. However, none of these clinical findings is, in itself, pathognomonic of KD. Many of these clinical features can be seen in other common febrile illnesses of children. It is for this reason that the diagnosis of KD is often considered to be a clinical challenge. Intravenous immunoglobulin along with aspirin is the gold standard therapy of treatment for KD. However, there is no consensus on the treatment of resistant forms of KD. Patients with KD should be on long-term follow-up especially if they have developed coronary artery abnormalities during the acute stage. This article reviews etiology, pathogenesis, clinic, diagnostics, and treatment options of Kawasaki disease.
Keywords: Kawasaki disease, vasculitis, Kawasaki syndrome, Mucocutaneous lymph node syndrome, intravenous immunoglobulin.