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.

Kawasaki%20disease Diagnosis


  • In the absence of a specific diagnostic test or pathognomonic feature, clinical criteria have been established to assist physicians in diagnosing Kawasaki Disease
  • Taking a careful history is necessary in children who lack a clear explanation for fever
  • Clinical features may not be present simultaneously, watchful waiting is sometimes necessary before a diagnosis can be made
  • Three clinical phases occur in the natural history of Kawasaki disease
    • Acute phase: 1st 2 weeks of illness; characterized by fever & other acute signs of illness
    • Subacute phase: 3rd & 4th week of illness; characterized by desquamation, thrombocytosis, development of coronary aneurysms & is associated w/ highest risk of sudden death in patients in whom aneurysms have developed
    • Convalescence phase: from 5th to 8th week since the onset of illness; all clinical signs of illness have disappeared & continues until erythrocyte sedimentation rate (ESR) & C-reactive protein (CRP) returns to normal level


Incomplete Kawasaki Disease

  • Patients w/ an inflammatory disorder who did not meet the clinical case definition but w/ persistent fever, at least 2 of the principal clinical features, & echocardiography revealing coronary artery abnormalities
  • More common in young infants <1 year of age
  • Accurate diagnosis & timely treatment is important due to substantial risk of developing coronary abnormalities
  • Lab findings similar to those of classic cases
  • These patients may also clinically display noncoronary cardiac lesions (eg pancarditis, conduction system abnormalities, subclinical ventricular dysfunction or subtle ventricular dilations) which are possibly independent of coronary artery abnormalities
  • Atypical Kawasaki disease refers to patients who have a problem (ie renal impairment) that generally is not seen in patients w/ Kawasaki disease
Refractory Kawasaki Disease
  • Approximately 10-20% of patients fail to respond to initial intravenous immunoglobulin (IVIG) treatment
    • Failure may be described as patients in whom inflammatory parameters do not subside & fever persists or recurs 24-48 hours after intravenous immunoglobulin (IVIG) infusion
  • Several factors may predict patient’s unresponsiveness to intravenous immunoglobulin (IVIG):
    • Low levels of sodium & albumin
    • Low platelet count
    • Day of illness at initial treatment
    • Neutrophil leukocytosis
    • High C-reactive protein (CRP) level & transaminases
    • Patient’s age
  • Patients who failed to respond to treatment have a higher risk of developing coronary artery abnormalities

Laboratory Tests

  • Even though nonspecific, can provide diagnostic support in patients w/ clinical features that are suggestive of Kawasaki disease
  • Certain lab findings are characteristic of Kawasaki disease
  • Complete blood count (CBC)
    • Neutrophil leukocytosis (predominance of immature & mature granulocytes)
    • Mild to moderate normochromic anemia [hemoglobin levels <2 standard deviation (SD) for age]
    • Platelet count rapidly increases w/in the next 2 weeks (ranges from 500,000 to >1 million/mm3)
      • A low platelet count at illness presentation is a risk factor for coronary aneurysm
      • Thrombocytopenia is seen rarely in the acute stage & may be a sign of disseminated intravascular coagulation
  • Inflammatory markers
    • Elevated erythrocyte sedimentation rate (ESR) & C-reactive protein (CRP), usually returns to normal by 6-10 weeks after the onset of illness
      • Erythrocyte sedimentation rate (ESR) often above 40 mm/hour; C-reactive protein (CRP) typically reaches levels of 3 mg/dL
      • C-reactive protein (CRP) is more accurate after intravenous immunoglobulin (IVIG) therapy since intravenous immunoglobulin (IVIG) elevates the erythrocyte sedimentation rate (ESR)
    • Elevated serum amyloid-A (SAA)
  • Mild to moderate elevations of serum transaminase may occur in ≤40% of patients
  • Mild hyperbilirubinemia in approximately 10%
    • Hypoalbuminemia is common & associated w/ more severe & prolonged acute disease
  • Urinalysis: proteinuria, leukocytosis, Intermittent mild to moderate sterile pyuria
  • Cerebrospinal fluid analysis
    • Approximately 50% demonstrate evidence of aseptic meningitis w/ predominance of mononuclear cells, as well as normal glucose & protein levels
  • Synovial fluid analysis
    • Purulent fluid w/ white blood cell count of 125,000-300,000/mm3
  • Plasma lipids are markedly altered; depressed plasma cholesterol, high density lipoprotein & apolipoprotein
  • Cardiac enzymes
    • Serum cardiac troponin I - marker specific for myocardial damage reported in acute Kawasaki Disease

Electrocardiogram (ECG) at rest

  • During acute phase, reveals findings suggestive of myocardial injury & abnormal repolarization: prolonged PR interval, deep Q waves, prolonged QT interval, low voltage, ST-T changes & arrhythmias

Holter Electrocardiogram (ECG)

  • Patients w/ stenosis or giant aneurysm should undergo holter electrocardiogram (ECG) recording at least once to determine whether ischemic findings are present or development of high-risk arrhythmias is possible
  • Used in patients complaining of frequent chest pain, chest discomfort &/or palpitations


  • W/ the exception of echocardiography, imaging studies are not performed routinely in suspected Kawasaki disease patients
  • Evaluation of cardiovascular sequelae requires serial cardiac ultrasound studies

Chest X-ray (X-ray)

  • Abnormalities are observed in about 15% of patients
  • Peribronchial cuffing or increased interstitial markings w/ occasional pulmonary nodules
  • Abnormal or enlarged heart shadow in patients w/ poor cardiac function due to chronic myocardial infarction (MI) & in patients w/ volume overload caused by mitral or aortic insufficiency
  • Presence of calcification of coronary aneurysm suggests the presence or progression of giant aneurysm or stenotic lesions
Echocardiography at rest
  • For uncomplicated cases, should be performed at the time of diagnosis, at 2 weeks & at 6-8 weeks after the onset of illness
  • More frequent echocardiographic evaluation is needed to guide management in children w/ higher risk
  • Ideal imaging modality for cardiac assessment
    • Has a high sensitivity & specificity for the detection of abnormalities of the proximal left main coronary artery (LMCA) & right coronary artery (RCA)
    • Frequent sites of coronary aneurysms are the proximal left anterior descending coronary artery, proximal right coronary artery & left main coronary artery (LMCA)
  • Can be used to evaluate coronary morphology over time to detect coronary dilatation specific to coronary artery lesions associated w/ Kawasaki disease
  • Can also determine the presence/absence of thrombi w/in aneurysms
  • Most useful method in evaluating cardiac function deterioration due to myocardial injury & the severity of valvular disease

Stress Echocardiography

  • Indicated to assess the existence & functional consequences of coronary artery abnormalities in children w/ Kawasaki disease & coronary aneurysm
  • Enables real time evaluation of left ventricular wall motion in patients during exercise (treadmill or ergometer) or w/ administration of Dobutamine or Dipyridamole

Other tests

  • Doppler color echocardiography
  • Magnetic resonance imaging (MRI) & magnetic resonance angiography (MRA)
  • Dual-source computed tomography (DSCT)
  • Coronary angiography
  • Intravascular ultrasound
  • Cardiac catheterization


  • Coronary artery aneurysms or ectasia develop in approximately 15 to 25% of untreated children & may lead to myocardial infarction (MI), ischemic heart disease or sudden death
  • Although damage of coronary vessels is the main complication of Kawasaki disease, systemic inflammation in other organs (eg myocardium, liver, lungs or kidneys) has been documented
  • Leading cause of acquired heart disease in children <5 years of age living in developed countries
Digital Edition
Asia's trusted medical magazine for healthcare professionals. Get your MIMS JPOG - Malaysia digital copy today!
Sign In To Download
Editor's Recommendations
Most Read Articles
01 Dec 2020
Tetanus toxoid 5 Lf, diphtheria toxoid 2 Lf, pertussis toxoid 2.5 mcg, filamentous haemagglutinin 5 mcg, fimbriae types 2 and 3 5 mcg, pertactin 3 mcg
Dr. Hsu Li Yang, Dr. Tan Thuan Tong, Dr. Andrea Kwa, 08 Jan 2021
Antimicrobial resistance has become increasingly dire as the rapid emergence of drug resistance, especially gram-negative pathogens, has outpaced the development of new antibiotics. At a recent virtual symposium, Dr Hsu Li Yang, Vice Dean (Global Health) and Programme Leader (Infectious Diseases), NUS Saw Swee Hock School of Public Health, presented epidemiological data on multidrug-resistant (MDR) gram-negative bacteria (GNB) in Asia, while Dr Tan Thuan Tong, Head and Senior Consultant, Department of Infectious Diseases, Singapore General Hospital (SGH), focused on the role of ceftazidime-avibactam in MDR GNB infections. Dr Andrea Kwa, Assistant Director of Research, Department of Pharmacy, SGH, joined the panel in an interactive fireside chat, to discuss challenges, practical considerations, and solutions in MDR gram-negative infections. This Pfizer-sponsored symposium was chaired by Dr Ng Shin Yi, Head and Senior Consultant of Surgical Intensive Care, SGH.
Pearl Toh, 26 Nov 2020
Inhaled corticosteroid (ICS) should be the mainstay of long-term asthma management — such is the key message of the latest Singapore ACE* Clinical Guidance (ACG) for asthma, released in October 2020.
Audrey Abella, 6 days ago
A pilot telemedicine initiative may be an alternative for facilitating delivery of intravenous iron (IVI) for individuals requiring iron supplementation.