### Date : 2024-12-21 19:14 ### Topic : Kawasaki Disease #pediatrics ---- ### **Kawasaki Disease** **Kawasaki disease** is a rare, acute, systemic **vasculitis** (inflammation of the blood vessels) that primarily affects **children**, particularly those under the age of 5. It is one of the most common causes of **acquired heart disease** in children, and the primary concern in the disease is the **risk of coronary artery aneurysms**. --- ### **Etiology and Pathophysiology** The exact cause of Kawasaki disease is unknown, but it is believed to involve an **immune-mediated response** triggered by an infection or other environmental factors in genetically predisposed individuals. The disease leads to inflammation of medium-sized blood vessels, including the **coronary arteries**, which supply blood to the heart muscle. The disease typically occurs in **episodes**, with inflammation affecting the blood vessels, skin, eyes, mouth, and other organs. This inflammation can lead to **long-term damage**, particularly to the heart. --- ### **Clinical Features** Kawasaki disease is characterized by a combination of **fever** and **inflammation of multiple organ systems**. The disease progresses through several stages, and the classic presentation includes the following **5 key criteria** (known as the **"Kawasaki Criteria**"): #### **1. Fever (≥5 days)**: - **High fever** lasting at least **5 days**, often unresponsive to typical antipyretics like acetaminophen. #### **2. Rash**: - A **polymorphous rash** (different types of rash), often involving the trunk, extremities, and **genital area**. - The rash may peel in the **desquamative phase** (when the skin begins to shed). #### **3. Conjunctivitis**: - **Bilateral, non-exudative conjunctivitis** (red, inflamed eyes) without pus or discharge. #### **4. Oral Changes**: - **Red, cracked lips** (often with fissures), **strawberry tongue** (a red, swollen tongue with prominent papillae), and redness inside the mouth. #### **5. Swelling and Redness of the Hands and Feet**: - **Swelling** and **erythema** (redness) of the **hands** and **feet**, often accompanied by peeling of the skin (especially on the fingers and toes) during the convalescent phase. #### **6. Cervical Lymphadenopathy**: - **Enlarged, tender lymph nodes**, typically on one side of the neck (unilateral). The **diagnosis** of Kawasaki disease is made if a patient meets **5 of the 6 criteria** and has a **fever** lasting more than 5 days. However, the disease can also be diagnosed with fewer criteria if there is evidence of **coronary artery involvement** or if the patient has **other symptoms** of Kawasaki disease. --- ### **Complications** The most serious complications of Kawasaki disease are **cardiovascular** in nature, particularly the **coronary arteries**: 1. **Coronary Artery Aneurysms**: - Inflammation can cause the walls of the coronary arteries to weaken, resulting in **aneurysm formation** (abnormal dilation of the arteries). - This can lead to **thrombosis** (blood clot formation), potentially causing **heart attacks** in children, which is the most serious and life-threatening complication of Kawasaki disease. 2. **Myocardial Infarction (Heart Attack)**: - Thrombosis in the coronary arteries can lead to **myocardial infarction** (heart attack), though this is rare. 3. **Valvular Regurgitation**: - Inflammation of the **heart valves** can lead to **valvular insufficiency** (leakage of the heart valve). 4. **Pericarditis**: - Inflammation of the **pericardium** (the lining around the heart) can occur. 5. **Aortic Involvement**: - **Aortic aneurysms** or **aortic regurgitation** may occur in some cases. --- ### **Diagnosis** Diagnosis of Kawasaki disease is primarily **clinical**, based on the presence of the **Kawasaki criteria**. However, several tests and imaging techniques are used to help monitor the progression of the disease and assess the risk of complications, especially in relation to the **heart**: 1. **Echocardiogram**: - A key diagnostic tool for assessing the condition of the **coronary arteries** and detecting **coronary artery aneurysms**. **Echocardiography** is often performed repeatedly to monitor for complications. 2. **Blood Tests**: - **Elevated inflammatory markers**: Such as **C-reactive protein (CRP)** and **erythrocyte sedimentation rate (ESR)**. - **Anemia**, **leukocytosis** (high white blood cell count), and **thrombocytosis** (high platelet count) are common findings. 3. **Urinalysis**: - **Mild proteinuria** or **sterile pyuria** (presence of white blood cells in the urine without infection) may be present. --- ### **Treatment** 1. **Intravenous Immunoglobulin (IVIG)**: - The mainstay of treatment is the administration of **IVIG**, which is effective in reducing **inflammation** and **preventing coronary artery complications** (such as aneurysms). IVIG is given as a **single high-dose infusion** within the first **10 days** of illness. 2. **Aspirin**: - **High-dose aspirin** is used initially to reduce inflammation and fever, followed by **low-dose aspirin** to help prevent blood clot formation. Aspirin should be continued until there is no evidence of **coronary artery abnormalities**. 3. **Corticosteroids** (optional): - In severe or resistant cases, **corticosteroids** may be added to the treatment regimen. However, their role in Kawasaki disease remains somewhat controversial and is used selectively. 4. **Antiplatelet Therapy**: - For patients with **coronary artery aneurysms** or other significant cardiovascular risk factors, **antiplatelet therapy** (like low-dose aspirin or clopidogrel) is used to prevent clot formation in the coronary arteries. 5. **Follow-up**: - Regular **echocardiograms** are needed to monitor for **coronary artery aneurysms** or other heart complications. In some cases, further interventions may be needed to manage coronary artery issues, including **angioplasty** or **stenting**. --- ### **Prognosis** - **With treatment**: Early treatment with IVIG significantly reduces the risk of **coronary artery complications** and improves long-term outcomes. **Most children** recover well, with many avoiding significant cardiovascular issues if treated promptly. - **Without treatment**: The risk of **coronary artery aneurysms**, **myocardial infarction**, and other severe complications is higher, which can lead to long-term health problems or even death. - **Long-term follow-up**: Children who have had Kawasaki disease should be **monitored** into adulthood for potential cardiovascular issues, including **coronary artery disease**, and should receive **routine cardiovascular evaluations**. --- ### **Conclusion** **Kawasaki disease** is an acute, systemic vasculitis that primarily affects children and can lead to **serious cardiovascular complications**, particularly **coronary artery aneurysms**. Early recognition and treatment with **intravenous immunoglobulin (IVIG)** and **aspirin** significantly improve outcomes and prevent long-term heart damage. Regular follow-up is essential to monitor for any residual cardiovascular issues, especially in patients with coronary artery involvement. ### Reference: - ### Connected Documents: -