Kawasaki disease is an acute, self-limited vasculitis predominantly affecting infants and children. It is characterized by fever, bilateral non-exudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy
Pathophysiology
The exact etiology of Kawasaki disease remains unknown, but it is suspected to involve an infectious agent due to its epidemicity and seasonality. The pathogenesis is closely associated with dysregulation of immune responses to various viruses or microbes
. The disease primarily affects the coronary arteries, leading to inflammation and potential aneurysm formation
Clinical course
Kawasaki disease is an acute febrile illness that can lead to coronary artery aneurysms if not treated early with high-dose intravenous immunoglobulin (IVIG)
. The disease is self-limited, with systemic inflammation subsiding over time, although coronary arteritis may persist, resulting in aneurysm formation
Prognosis
The prognosis of Kawasaki disease is generally good, especially with early treatment. The risk of coronary artery aneurysms is reduced fivefold if IVIG is administered within 10 days of fever onset
. However, the risk of myocardial infarction from coronary artery thrombosis is greatest in the first two years after illness onset, and stenosis and occlusion can progress over years
Risk factors
Risk factors for an unfavorable outcome include male gender, absence of immunoglobulin treatment in the acute phase, and atypical age for Kawasaki disease (e.g., older than 10 years)
. Additionally, certain genetic backgrounds may leave individuals more susceptible to Kawasaki disease
In conclusion, Kawasaki disease is an acute, self-limited vasculitis primarily affecting children, with a generally good prognosis if treated early. The exact cause remains unknown, but it is suspected to involve an infectious agent. Risk factors for an unfavorable outcome include male gender, absence of immunoglobulin treatment in the acute phase, and atypical age for the disease.