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| Disease found: | Kawasaki Disease |
| Current as of: | October 8, 2024 |
| Disease Overview: | Small-to-medium vessel vasculitis, most prominently affecting coronary arteries with multisystem inflammation. [more info] Etiology is unknown but theorized to be abnormal immune system activity after an infection. Most common in children of Asian descent. [more info]] |
| Signs and Symptoms: | Triphasic disease course. Acute phase: high-spiking fever for 1-2 weeks, may have conjunctivitis, lymphadenopathy, oropharyngeal inflammation. Subacute phase: 4 weeks of desquamation, arthralgias, myositis, risk of dangerous coronary artery aneurysm. Convalecent phase for 4-8 weeks with remaining risk of coronary artery aneurysm. [more info] |
| Diagnosis: | Diagnosis is clinical; there is no specific test. Fever >4 days with at least 4 of 5 key symptoms (reddened eyes; changes of the lips and mouth; reddish, swollen extremities; rash; and swollen lymph nodes) and ruling out infectious causes. ECG and echocardiography are needed to assess for cardiac involvement. [more info] |
| Treatment: | IVIG within 10 days of fever can help prevent life-threatening cardiac involvement. Aspirin should be provided high-dose until patient is afebrile, low-dose until patient enters convalescent phase (benefits of aspirin outweigh risk of Reye syndrome). [more info]. |
| Clinical Management: | Echocardiogram must be performed minimum at baseline and 6-8 weeks after illness onset; more frequent cardiac monitoring if cardiac involvement is seen. Cardiac assessment should be performed every 6mo-5yr indefinitely, depending on extent of damage seen. If aneurysms are present, more prolonged anticoagulation therapy should be considered. Severe cases may require cardiac surgery. Influenza vaccine should be given to children on prolonged aspirin to minimize Reye syndrome risk. [more info] |
| Referral: | Pediatric cardiology should be consulted to manage care plan.Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health. |
| Clinical Trials: | Two US trials are currently recruiting; several additional trials recruiting in France and China. |