Abstract
Kawasaki Disease (KD) is a self-limiting systemic vasculitis common among children under five years of age. Diagnosis of the disease is made primarily from the clinical features presented during the illness. Coronary artery aneurysm (CAA) is the main complication of this disease which can be prevented largely by the early diagnosis and administration of IVIG (Intravenous Immunoglobulin). Even with first dose of IVIG up to 10% - 20% of patients develop refractory Kawasaki Disease, i.e., remain unresponsive to initial IVIG treatment. Second dose of IVIG is recommended treatment for refractory Kawasaki Disease but other alternative treatments are also being considered to lower the risk of complications. Different drugs are still in trial phase and some others have limited studies on larger population. Most of the study reports that involved newer drugs have limited patients or are from single centers which are very hard to apply to larger population. We will review general treatment approach in refractory Kawasaki disease.
Highlights
Kawasaki disease is an acute, self-resolving systemic vasculitis [1], that is commonly seen in children under the age of five years
Before the term Kawasaki disease (KD), it was reported as syndrome of mucocutaneous lymph node with infantile polyarteritis nodosa which was common among younger Asian population
Diagnostic criteria [4] of Kawasaki consist of fever lasting for at least five days along with any four among these 5 clinical features, 1) Bilateral bulbar conjunctival injection; 2) Oral mucous membrane changes, including injected or fissured lips, injected pharynx, or strawberry tongue; 3) Peripheral extremity changes, including erythema of palms or soles, edema of hands or feet, and periungual desquamation; 4) Polymorphous rash; and 5) Cervical lymphadenopathy. Patient fulfilling these criteria are diagnosed with complete Kawasaki disease and those not fulfilling these criteria but having 2 or 3 criteria and a strong clinical suspicion of Kawasaki Disease (KD) [5] can further be evaluated with the help of laboratory and echocardiographic evaluation and if further criteria fulfilled those are diagnosed as incomplete Kawasaki disease
Summary
Kawasaki disease is an acute, self-resolving systemic vasculitis [1], that is commonly seen in children under the age of five years. Before the term Kawasaki disease (KD), it was reported as syndrome of mucocutaneous lymph node with infantile polyarteritis nodosa which was common among younger Asian population. This disease primarily affects medium sized arteries which complicate into acquired childhood cardiac disease. Kawasaki disease has replaced acute rheumatic fever as leading cause of childhood acquired cardiac disease in Japan, the United States and in Europe [3]
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