kawasaki%20disease
KAWASAKI DISEASE
Kawasaki disease is an acute, febrile illness that is self-limited. It is a systemic vasculitic syndrome that primarily involves the medium- and small-sized muscular arteries of the body.
It is also known as mucocutaneous lymph node syndrome.
It affects primarily children <5 years old with peak incidence in 1-2 year of age.
The cause remains unknown but current research supports an infectious origin.
Epidemiological findings suggest that genetic predisposition and environmental factors play a role in the pathogenesis of the disease.

Monitoring

Long-Term Follow-Up

W/o Detectable Coronary Lesions

  • Children w/ no known cardiac sequelae during the 1st month of Kawasaki disease appear to return to their previous state of health, w/o signs or symptoms of cardiac impairment
  • Altered lipid metabolism persists beyond clinical resolution of the disease
  • Lower myocardial flow reserve & higher total coronary resistance have been found in those w/ history of Kawasaki disease w/ normal epicardial coronary arteries

Regression of Coronary Lesions

  • Coronary artery lesions resulting from Kawasaki disease change dynamically w/ time
  • The likelihood that an aneurysm will resolve appears to be determined by:
    • Initial size: smaller aneurysms have a greater likelihood of regression
    • <1 year of age at onset of the disease
    • Fusiform morphology
    • Location in distal coronary segment

Persistent Coronary Artery Abnormalities

  • Stenotic lesions that are secondary to marked myointimal proliferation are frequently progressive
  • Highest rate of progression to stenosis occurs in patients w/ large aneurysms
  • Worst prognosis occurs in children w/ giant aneurysms since thrombosis is promoted by sluggish blood flow w/in massively dilated vascular space & the frequent occurrence of stenotic lesions at the proximal or distal end of the aneurysms

Cardiovascular Risk Stratification

  • Stratification of patients according to their relative risk of myocardial ischemia allows for patient management to be individualized
  • The risk level for a given patient w/ coronary artery involvement may change over time because of the changes in coronary artery morphology
Risk Level I - Patients w/o coronary artery changes on echocardiography at any stage of the illness
  • No antiplatelet therapy nor restriction of physical activity is necessary beyond the initial 6-8 weeks after the onset of illness; dilatation subsides w/in 30 days after onset
  • Suggest periodic assessment & counseling about known cardiovascular risk factors every 5 years

Risk Level II - Patients w/ transient coronary artery ectasia or dilatation, which disappears w/in the initial 6-8 weeks after the onset of illness

  • No antiplatelet therapy nor restriction of physical activity is necessary beyond the initial 6-8 weeks after the onset of illness
  • Recommend risk assessment & counseling at 3-5 year intervals

Risk Level III - Patients w/ solitary small-to-medium (>3 mm but <6 mm) coronary artery aneurysm in >1 coronary arteries on echocardiography or angiography, & aneurysm persists or worsens beyond 30 days after onset

  • Long term antiplatelet therapy w/ low-dose Aspirin should be administered until the aneurysms regress
  • No restriction on physical activity in patients <11 years of age
  • For patients 11-20 years of age, stress tests w/ myocardial perfusion evaluation may be useful to guide recommendations for physical activity
    • Due to risk of bleeding, participation in competitive collision or high-impact sports is discouraged in children receiving antiplatelet therapy
  • Annual follow-up by a pediatric cardiologist w/ echocardiogram (ECG) is recommended
    • Stress test w/ myocardial perfusion imaging is recommended every 2 years in patients >10 years of age
  • Coronary angiography is indicated if myocardial ischemia is demonstrated by non-invasive tests

Risk Level IV - Patients w/ ≥1 large coronary artery aneurysm (≥6 mm) including giant aneurysms & patients in whom a coronary artery contains multiple (segmented) or complex aneurysms w/o obstruction

  • Long term antiplatelet therapy is recommended
    • Adjunctive therapy w/ Warfarin w/ a target International Normalized Ratio (INR) of 2-2.5 is recommended for patients w/ giant aneurysms
    • Low molecular weight Heparin may be considered as an alternative to Warfarin for infants & toddlers
  • Recommendation about physical activity should be guided by annual stress tests w/ myocardial perfusion evaluation
    • Collision or high-impact sports should be discouraged
    • Participation in non contact dynamic or recreational sports is encouraged if there is no evidence of stress-induced myocardial ischemia
  • Cardiology evaluation w/ echocardiogram (ECG) should be done at 6 months intervals
  • Stress tests w/ myocardial perfusion evaluation should be performed annually
  • Monitor for known risk factors of atherosclerosis; counsel family accordingly
  • Cardiac catheterization w/ selective coronary angiography should be performed 6-12 months after recovery from the acute illness, or sooner if clinically indicated
  • Follow-up angiography may be indicated if noninvasive studies suggest myocardial ischemia
  • For females of childbearing age, reproductive counseling is strongly recommended

Risk Level V - Patients w/ coronary artery obstruction confirmed by angiography

  • Long-term antiplatelet therapy, w/ or w/o adjunctive therapy w/ Warfarin anticoagulation, is recommended
  • Beta-blockers should be considered to reduce myocardial oxygen consumption
  • Recommendation about dynamic physical activities should be based on the patient’s response to stress testing
    • Collision or high-impact sports should be discouraged
  • Patients should avoid a sedentary lifestyle
  • Cardiology evaluation w/ an echocardiogram (ECG) should be obtained at 6 months intervals
  • Stress tests w/ myocardial perfusion evaluation should be performed annually
  • Monitor patient for known risk factors of atherosclerosis; counsel family accordingly
  • Cardiac catheterization w/ selective coronary angiography is recommended to address the therapeutic options of bypass grafting or catheter intervention & to identify the extent of collateral perfusion
  • Repeat cardiac catheterization may be indicated when new onset or worsening myocardial ischemia is suggested by noninvasive diagnostic testing or clinical presentation
  • For females of childbearing age, reproductive counseling is strongly recommended

Prognosis

  • Depends on cardiac involvement & its long-term sequelae
  • Coronary artery aneurysms develop in 20-40% of untreated children or in patients treated w/ only Aspirin, which may eventually lead to rupture in adulthood, ischemic heart disease & myocardial infarction (MI)
  • Aneurysms might be demonstrated in other extraparenchymal muscular arteries except the central nervous system (CNS) arteries

Prevention

  • Schedule for administration of inactivated childhood vaccines should not be interrupted
  • It may be necessary to reschedule administration of live viral vaccines (eg measles, varicella-containing vaccines) to at least 9-11 months after intravenous immunoglobulin (IVIG) administration
    • If the risk of exposure to measles is high, patients may be vaccinated earlier & then reimmunized ≥11 months after intravenous immunoglobulin (IVIG) administration if the patient has an inadequate serological response
  • Annual influenza vaccination is recommended in patients who are on long-term therapy w/ Aspirin to reduce the risk of Reye’s syndrome
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